Enlarged Spleen: Causes, Symptoms, and Treatments

What is the spleen and what causes an enlarged spleen (splenomegaly)?

The spleen sits under your rib cage in the upper left part of your abdomen toward your back. It is an organ that is part of the lymph system and works as a drainage network that defends your body against infection.

White blood cells produced in the spleen engulf bacteria, dead tissue, and foreign matter, removing them from the blood as blood passes through it. The spleen also maintains healthy red and white blood cells and platelets; platelets help your blood clot. The spleen filters blood, removing abnormal blood cells from the bloodstream.

A spleen is normally about the size of your fist. A doctor usually can’t feel it during an exam. But diseases can cause it to swell and become many times its normal size. Because the spleen is involved in many functions, many conditions may affect it.

An enlarged spleen is not always a sign of a problem. When a spleen becomes enlarged, though, it often means it has been doing its job but has become overactive. For example, sometimes the spleen is overactive in removing and destroying blood cells. This is called hypersplenism. It can happen for many reasons, including problems with too many platelets and other disorders of the blood.

Causes of an Enlarged Spleen

An enlarged spleen can be caused by infections, cirrhosis and other liver diseases, blood diseases characterized by abnormal blood cells, problems with the lymph system, or other conditions.

Here are some common causes of an enlarged spleen:

Infections

• Viral infections, such as mononucleosis
• Parasitic infections, such as toxoplasmosis
• Bacterial infections, such as endocarditis (an infection of your heart’s valves)

Cancer

Other causes of an enlarged spleen include:

• Inflammatory diseases such as sarcoidosis, lupus, and rheumatoid arthritis
• Trauma, such as an injury during contact sports
• Cancer that has spread (metastasized) to the spleen
• A cyst, a noncancerous fluid-filled sac
• A large abscess, a pus-filled cavity usually caused by a bacterial infection
• Infiltrative diseases such as Gaucher disease, amyloidosis, or glycogen storage diseases

Symptoms of an Enlarged Spleen

Most people don’t know they have an enlarged spleen because symptoms are rare. People usually find out about it during a physical exam. These are the most common symptoms of an enlarged spleen:

• Being unable to eat a large meal.
• Feeling discomfort, fullness, or pain on the upper left side of the abdomen; this pain may spread to your left shoulder.

If you have pain that is severe or gets worse when taking a deep breath, see your doctor right away.

If you have an enlarged spleen, you may develop other signs or symptoms, too. These are related to the underlying disease. They may include signs and symptoms such as:

Your doctor will ask you questions and do a physical exam to diagnose an enlarged, painful spleen. This involves palpating (examining by touch) your spleen. You will also likely need diagnostic tests to confirm the cause of the swollen spleen. These may include blood tests, an ultrasound, or computerized tomography (CT) scan. In some cases, other tests may be needed.

Treatments for an Enlarged Spleen

Limit any activities that could rupture your spleen, such as contact sports. A ruptured spleen can cause lots of blood loss and be life threatening. It’s important to seek treatment for the cause of your enlarged spleen. Left untreated, an enlarged spleen can lead to serious complications. In most cases, treatment of the underlying cause of the enlarged spleen can prevent removal of the spleen. In some cases, the spleen will need to be removed surgically (splenectomy).

If surgery is needed, a surgeon is likely to remove the spleen using laparoscopy rather than open surgery. This means the surgery is performed through small incisions. A laparoscope allows the surgeon to view and remove the spleen.

If your spleen is removed, you cannot effectively clear certain bacteria from your body and will be more vulnerable to certain infections. So vaccines or other medications are needed to prevent infection.

Gaucher Disease: Causes, Symptoms, and Treatment

What Is Gaucher Disease?

If you just found out that you or your child has Gaucher disease, it’s natural to have lots of questions and concerns about what to expect. It’s a rare condition that can cause a variety of symptoms, from weakening of your bones to easy bruising.

Although there’s no cure, it’s important to keep in mind that depending on which type you have, treatment can help you manage some of the symptoms.

Gaucher causes problems with the way your body gets rid of a certain kind of fat. With all types of this disease, an enzyme you need to break it down doesn’t work right. The fat builds up, especially in your liver, spleen, and bone marrow, causing problems.

There are three main types of Gaucher disease. Type 1 is the most common. If you have it, your symptoms may be mild, moderate, or severe, but some people don’t notice any problems at all. There are several treatments for type 1.

Type 2 and type 3 are more serious. Type 2 affects the brain and spinal cord. Babies with type 2 usually don’t live past age 2. Type 3 also causes damage to the brain and spinal cord, but symptoms usually show up later in childhood.

Gaucher disease can have many symptoms, including a swollen belly, bruising, and bleeding.

Your blood might not clot well, or you might get anemia. It can also cause bone mineral loss that leads to pain and easily broken bones.

It’s passed along in families — about 1 in 450 people of Ashkenazi Jewish descent (from Eastern and Central Europe) have the condition. It’s the most common genetic disease that affects Jewish people.

Whether you have the disease or you’re the parent of a child who has it, it’s important to get support to help you face the challenges ahead. Reach out to your family and friends to get the backing you need. Ask your doctor about how to join a support group, where you can meet other people who are going through the same things you are.

Causes

Gaucher disease isn’t something you “catch,” like a cold or the flu. It’s an inherited condition that’s caused by a problem with the GBA gene.

You get the disease when both of your parents pass along a damaged GBA gene to you. You can pass a faulty gene on to your children even if you don’t have Gaucher disease.

Symptoms

How you feel depends on what type of Gaucher disease you’ve got.

Type 1. It’s the most common form. It usually doesn’t affect your brain or spinal cord.

Symptoms of type 1 can appear at any time in your life, but they usually show up by the teen years. Sometimes the disease is mild and you won’t notice any problems.

Some symptoms you might have are:

• Easy bruising
• Nosebleeds
• Fatigue
• Enlarged spleen or liver, which makes your belly look swollen
• Bone problems like pain, breaks, or arthritis

Type 2. It affects the brain and spinal cord and is very serious. Babies with this form usually don’t live past age 2. The symptoms include:

• Slow back-and-forth eye movement
• Not gaining weight or growing as expected, called “failure to thrive”
• High-pitched sound when breathing
• Seizures
• Brain damage, especially to the brain stem
• Enlarged liver or spleen

Type 3. This type also affects the brain and spinal cord. The symptoms are similar to type 2, but they usually show up later in childhood and take longer to get worse.

Perinatal lethal Gaucher disease. This is the most severe type. An infant with this form usually lives only a few days. These symptoms are overwhelming for a newborn:

• Too much fluid in the baby’s body before or soon after they’re born
• Dry, scaly skin and other skin problems
• Enlarged liver and spleen
• Severe brain and spinal cord problems

Cardiovascular Gaucher disease (type 3C). This is rare and mainly affects your heart. You might have symptoms like:

• Hardening of the heart valves and blood vessels
• Bone disease
• Enlarged spleen
• Eye problems

Getting a Diagnosis

When you go to your doctor, they might ask:

• When did you notice your symptoms?
• What’s your family’s ethnic background?
• Have past generations of your family had similar medical problems?
• Did any children in your extended family pass away before age 2?

If your doctor thinks it’s Gaucher disease, they can confirm that with a blood or saliva test. They’ll also do tests regularly to track the condition. You may have an MRI (magnetic resonance imaging) scan to see if your liver or spleen is swollen. You might also get a bone density test to check for any loss of bone.

When Gaucher disease runs in your family, blood or saliva tests can help you find out if you could pass the disease to your child.

Questions for Your Doctor

• Will my symptoms change over time? If so, how?
• Which treatments are best for me?
• Do these treatments have side effects? What can I do about them?
• Is there a clinical trial that could help?
• Are there new symptoms I should watch for?
• How often do I need to see you?
• Are there other specialists I should see?
• Does having this disease put me at risk for any others?
• Should I add my name to a Gaucher disease registry?
• If I have more children, what are the chances they’ll have Gaucher disease?

Treatment

How you treat it depends on what type of Gaucher disease you have.

Enzyme replacement therapy is one option for people who have type 1 and some with type 3. It helps reduce anemia and shrinks an enlarged spleen or liver.

Enzyme replacement therapy medications may include:

Other medications for type 1 are eliglustat (Cerdelga) and miglustat (Zavesca). They are pills that curb the process in your body that forms the fatty materials when you have an enzyme shortage.

There’s no treatment that can stop type 3 from causing damage to the brain, but researchers are always looking for new ways to fight the disease.

Other treatments that can help your symptoms are:

• Blood transfusions for anemia
• Medications to strengthen your bones, prevent the buildup of fat, and ease pain
• Joint replacement surgery to help you move better
• Surgery to remove a swollen spleen
• Stem cell transplant to reverse type 1 symptoms. This procedure is complicated and can cause both short-term and long-term problems, so it’s rarely used.

What to Expect

Because the disease is different for each person, you’ll need to work with your doctor to make sure you’re getting the right care. Treatment can help you feel better and might help you live longer.

You may want to consider joining a clinical trial. Scientists use them to study new methods to treat Gaucher disease. These trials test new drugs to see if they’re safe and if they work. They often are a way for people to try new medicine that isn’t available to everyone. Your doctor can tell you if one of these trials might be a good fit for you.

If your child has Gaucher, they might not grow as quickly as other kids. They might be late in reaching puberty.

Depending on your symptoms, you may need to avoid contact sports or limit your activity. Some people have severe pain and fatigue. It may take extra effort to be active. Little things can make a big difference, though. For example, wheelchairs or crutches can help when you’re having trouble walking. Naps are also useful.

If you have severe symptoms, you may need regular help and might not be able to live alone.

Whether you have the disease or are caring for someone who has it, this is a tough condition to manage. When you get the support of others who face the same challenges, it can help you feel less isolated. Sharing information and tips can help make your daily life easier.

Think about getting professional counseling. It might also help you manage your feelings and frustrations about living with Gaucher disease.

Getting Support

You can get information on resources and support groups from the National Gaucher Foundation.

The organization has a mentor program that lets you connect with others who have the disease to share experiences and ideas for living with your condition.

Splenomegaly: Practice Essentials, Etiology, Epidemiology

Author

Neetu Radhakrishnan, MD Medical Oncologist, Kettering Cancer Care

Neetu Radhakrishnan, MD is a member of the following medical societies: American College of Physicians, American Society of Clinical Oncology, American Society of Hematology

Disclosure: Nothing to disclose.

Coauthor(s)

Ronald A Sacher, MBBCh, FRCPC, DTM&H Professor of Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center

Ronald A Sacher, MBBCh, FRCPC, DTM&H is a member of the following medical societies: American Association for the Advancement of Science, American Association of Blood Banks, American Clinical and Climatological Association, American Society for Clinical Pathology, American Society of Hematology, College of American Pathologists, International Society of Blood Transfusion, International Society on Thrombosis and Haemostasis, Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

Gina M Matacia-Murphy, MD Fellow in Hematology/Oncology, University of Cincinnati College of Medicine

Gina M Matacia-Murphy, MD is a member of the following medical societies: American Society of Clinical Oncology

Disclosure: Nothing to disclose.

Acknowledgements

Wadie F Bahou, MD Chief, Division of Hematology, Hematology/Oncology Fellowship Director, Professor, Department of Internal Medicine, State University of New York at Stony Brook

Wadie F Bahou, MD is a member of the following medical societies: American Society of Hematology

Disclosure: Nothing to disclose.

David Coffman, MD Fellow, Department of Surgery, Division of Trauma and Critical Care, Yale University School of Medicine

webmd.com”>Disclosure: Nothing to disclose.

Marcel E Conrad, MD Distinguished Professor of Medicine (Retired), University of South Alabama College of Medicine

Marcel E Conrad, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association of Blood Banks, American Chemical Society, American College of Physicians, American Physiological Society, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Association of Military Surgeons of the US, International Society of Hematology, Society for Experimental Biology and Medicine, and Southwest Oncology Group

Disclosure: No financial interests None None

Emmanuel N Dessypris, MD Professor of Medicine, Medical College of Virginia; Chief, Medical Service, Hunter Holmes McGuire Department of Veterans Affairs Medical Center

webmd.com”>Emmanuel N Dessypris, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Physicians, American Society of Hematology, New York Academy of Sciences, Society for Experimental Biology and Medicine, and Southern Society for Clinical Investigation

Disclosure: Nothing to disclose.

David J Draper, MD Fellow, Department of Hematology/Oncology, The University Hospital, University of Cincinnati College of Medicine

Disclosure: Nothing to disclose.

Lewis J Kaplan, MD, FACS, FCCM, FCCP Director, SICU and Surgical Critical Care Fellowship, Associate Professor, Department of Surgery, Section of Trauma, Surgical Critical Care, and Surgical Emergencies, Yale University School of Medicine

webmd.com”>Lewis J Kaplan, MD, FACS, FCCM, FCCP is a member of the following medical societies: American Association for the Surgery of Trauma, American College of Surgeons, Association for Academic Surgery, Association for Surgical Education, Connecticut State Medical Society, Eastern Association for the Surgery of Trauma, International Trauma Anesthesia and Critical Care Society, Society for the Advancement of Blood Management, Society of Critical Care Medicine, and Surgical Infection Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Enlarged spleen (splenomegaly) // Middlesex Health

Overview

Your spleen is an organ that sits just below your left rib cage. Many conditions — including infections, liver disease and some cancers — can cause an enlarged spleen. An enlarged spleen is also known as splenomegaly (spleh-no-MEG-uh-lee).

An enlarged spleen usually doesn’t cause symptoms. It’s often discovered during a routine physical exam. A doctor usually can’t feel the spleen in an adult unless it’s enlarged. Imaging and blood tests can help identify the cause of an enlarged spleen.

Treatment for an enlarged spleen depends on what’s causing it. Surgery to remove an enlarged spleen usually isn’t needed, but sometimes it’s recommended.

The spleen is a small organ usually about the size of your fist. But a number of conditions, including liver disease and some cancers, can cause your spleen to become enlarged.

Symptoms

An enlarged spleen typically causes no signs or symptoms, but sometimes it causes:

• Pain or fullness in the left upper belly that can spread to the left shoulder
• A feeling of fullness without eating or after eating a small amount because the spleen is pressing on your stomach
• Low red blood cells (anemia)
• Frequent infections
• Bleeding easily

When to see a doctor

See your doctor promptly if you have pain in your left upper belly, especially if it’s severe or the pain gets worse when you take a deep breath.

Causes

A number of infections and diseases can cause an enlarged spleen. The enlargement might be temporary, depending on treatment. Contributing factors include:

• Viral infections, such as mononucleosis
• Bacterial infections, such as syphilis or an infection of your heart’s inner lining (endocarditis)
• Parasitic infections, such as malaria
• Cirrhosis and other diseases affecting the liver
• Various types of hemolytic anemia — a condition characterized by early destruction of red blood cells
• Blood cancers, such as leukemia and myeloproliferative neoplasms, and lymphomas, such as Hodgkin’s disease
• Metabolic disorders, such as Gaucher disease and Niemann-Pick disease
• Pressure on the veins in the spleen or liver or a blood clot in these veins
• Autoimmune conditions, such as lupus or sarcoidosis

How the spleen works

Your spleen is tucked below your rib cage next to your stomach on the left side of your belly. Its size generally relates to your height, weight and sex.

This soft, spongy organ performs several critical jobs, such as:

• Filtering out and destroying old, damaged blood cells
• Preventing infection by producing white blood cells (lymphocytes) and acting as a first line of defense against disease-causing organisms
• Storing red blood cells and platelets, which help your blood clot

An enlarged spleen affects each of these jobs. When it’s enlarged, your spleen may not function as usual.

Risk factors

Anyone can develop an enlarged spleen at any age, but certain groups are at higher risk, including:

• Children and young adults with infections, such as mononucleosis
• People who have Gaucher disease, Niemann-Pick disease, and several other inherited metabolic disorders affecting the liver and spleen
• People who live in or travel to areas where malaria is common

Complications

Potential complications of an enlarged spleen are:

• Infection. An enlarged spleen can reduce the number of healthy red blood cells, platelets and white cells in your bloodstream, leading to more frequent infections. Anemia and increased bleeding also are possible.
• Ruptured spleen. Even healthy spleens are soft and easily damaged, especially in car crashes. The possibility of rupture is much greater when your spleen is enlarged. A ruptured spleen can cause life-threatening bleeding in your belly.

Diagnosis

An enlarged spleen is usually detected during a physical exam. Your doctor can often feel it by gently examining your left upper belly. However, in some people — especially those who are slender — a healthy, normal-sized spleen can sometimes be felt during an exam.

Your doctor might order these tests to confirm the diagnosis of an enlarged spleen:

• Blood tests, such as a complete blood count to check the number of red blood cells, white blood cells and platelets in your system and liver function
• Ultrasound or CT scan to help determine the size of your spleen and whether it’s crowding other organs
• MRI to trace blood flow through the spleen

Finding the cause

Sometimes more testing is needed to find the cause of an enlarged spleen, including a bone marrow biopsy exam.

A sample of solid bone marrow may be removed in a procedure called a bone marrow biopsy. Or you might have a bone marrow aspiration, which removes the liquid portion of your marrow. Both procedures might be done at the same time.

Liquid and solid bone marrow samples are usually taken from the pelvis. A needle is inserted into the bone through an incision. You’ll receive either a general or a local anesthetic before the test to ease discomfort.

A needle biopsy of the spleen is rare because of the risk of bleeding.

Your doctor might recommend surgery to remove your spleen (splenectomy) for diagnostic purposes when there’s no identifiable cause for the enlargement. More often, the spleen is removed as treatment. After surgery to remove it, the spleen is examined under a microscope to check for possible lymphoma of the spleen.

Treatment

Treatment for an enlarged spleen focuses on the what’s causing it. For example, if you have a bacterial infection, treatment will include antibiotics.

Watchful waiting

If you have an enlarged spleen but don’t have symptoms and the cause can’t be found, your doctor might suggest watchful waiting. You see your doctor for reevaluation in 6 to 12 months or sooner if you develop symptoms.

Spleen removal surgery

If an enlarged spleen causes serious complications or the cause can’t be identified or treated, surgery to remove your spleen (splenectomy) might be an option. In chronic or critical cases, surgery might offer the best hope for recovery.

Elective spleen removal requires careful consideration. You can live an active life without a spleen, but you’re more likely to get serious or even life-threatening infections after spleen removal.

Reducing infection risk after surgery

After spleen removal, certain steps can help reduce your risk of infection, including:

• A series of vaccinations before and after the splenectomy. These include the pneumococcal (Pneumovax 23), meningococcal and haemophilus influenzae type b (Hib) vaccines, which protect against pneumonia, meningitis and infections of the blood, bones and joints. You’ll also need the pneumococcal vaccine every five years after surgery.
• Taking penicillin or other antibiotics after your surgery and anytime you or your doctor suspects the possibility of an infection.
• Calling your doctor at the first sign of a fever, which could indicate an infection.
• Avoiding travel to parts of the world where certain diseases, such as malaria, are common.

Lifestyle and home remedies

Avoid contact sports — such as soccer, football and hockey — and limit other activities as recommended to reduce the risk of a ruptured spleen.

It’s also important to wear a seat belt. If you’re in a car accident, a seat belt can help protect your spleen.

Finally, be sure to keep your vaccinations up to date because your risk of infection is increased. That means at least an annual flu shot, and a tetanus, diphtheria and pertussis booster every 10 years. Ask your doctor if you need other vaccines.

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Enlarged spleen (splenomegaly) | Sparrow

An enlarged spleen is usually detected during a physical exam. Your doctor can often feel it by gently examining your left upper abdomen. However, in some people — especially those who are slender — a healthy, normal-sized spleen can sometimes be felt during an exam.

Your doctor may confirm the diagnosis of an enlarged spleen with one or more of these tests:

• Blood tests, such as a complete blood count to check the number of red blood cells, white blood cells and platelets in your system
• Ultrasound or computerized tomography (CT) scan to help determine the size of your spleen and whether it’s crowding other organs
• Magnetic resonance imagining (MRI) to trace blood flow through the spleen

Imaging tests aren’t always needed to diagnose an enlarged spleen. But if your doctor recommends imaging, you generally don’t need any special preparation for an ultrasound or MRI.

If you’re having a CT scan, however, you may need to refrain from eating before the test. If you need to prepare, your doctor will let you know well in advance.

Finding the cause

Sometimes you may need more testing to find the cause of an enlarged spleen, including liver function tests and a bone marrow exam. These tests can provide more-detailed information about your blood cells than can blood drawn from a vein.

A sample of solid bone marrow is sometimes removed in a procedure called a bone marrow biopsy. Or you may have a bone marrow aspiration, which removes the liquid portion of your marrow. In many cases, both procedures are performed at the same time (bone marrow exam).

Both the liquid and solid bone marrow samples are usually taken from the pelvis. A needle is inserted into the bone through an incision. You’ll receive either general or local anesthesia before the test to ease discomfort.

A needle biopsy of the spleen is very rare because of the risk of bleeding.

Occasionally, your doctor may recommend surgery to remove your spleen when there’s no identifiable cause for the enlargement. After surgical removal, the spleen is examined under a microscope to check for possible lymphoma of the spleen.

Treatment

Treatment for an enlarged spleen focuses on the underlying problem. For example, if you have a bacterial infection, treatment will include antibiotics.

Watchful waiting

If you have an enlarged spleen but don’t have any symptoms and the cause can’t be found, your doctor may suggest watchful waiting. You’ll have to see your doctor for reevaluation in six to 12 months or sooner if you develop any symptoms.

Spleen removal surgery

If an enlarged spleen causes serious complications or the cause can’t be identified or treated, surgical removal of your spleen (splenectomy) may be an option. In chronic or critical cases, surgery may offer the best hope for recovery.

Elective spleen removal requires careful consideration. You can live an active life without a spleen, but you’re more likely to contract serious or even life-threatening infections after spleen removal. Sometimes radiation can shrink your spleen so that you can avoid surgery.

Reducing infection risk after surgery

After spleen removal, certain steps can help reduce your risk of infection, including:

• A series of vaccinations both before and after the splenectomy. These include the pneumococcal (Pneumovax 23), meningococcal and haemophilus influenzae type b (Hib) vaccines, which protect against pneumonia, meningitis and infections of the blood, bones and joints. You’ll also need the pneumococcal vaccine every five years after surgery.
• Taking penicillin or other antibiotics after your operation and anytime you or your doctor suspects the possibility of an infection.
• Calling your doctor at the first sign of a fever, because this may indicate an infection.
• Avoiding travel to parts of the world where certain diseases, such as malaria, are common.

Lifestyle and home remedies

Avoid contact sports — such as soccer, football and hockey — and limit other activities as recommended by your doctor. Modifying your activities can reduce the risk of a ruptured spleen.

It’s also important to wear a seat belt. If you’re in a car accident, a seat belt can help prevent injury to your spleen.

Finally, be sure to keep your vaccinations up to date because your risk of infection is increased. That means at least an annual flu shot, and a tetanus, diphtheria and pertussis booster every 10 years. Ask your doctor if you need any additional vaccines.

Frontiers | Splenomegaly in Children and Adolescents

Introduction

Splenomegaly is not regarded as a disease of its own, but rather as a potential symptom associated with different disorders. In healthy individuals a spleen is usually not palpable in most cases. A patient exhibiting splenomegaly may therefore present with a number of clinical signs and laboratory or imaging findings that are commonly associated with distinct diseases ranging from self-limiting benign to infectious disorders or even malignancies. Thus, the finding of an enlarged spleen in a patient should be investigated properly to ascertain the etiology which may represent a diagnostic challenge in some cases.

Establishing a practical clinical definition of splenomegaly is not trivial and also there is no generally accepted grading of splenomegaly. Length or weight of the organ are typically used to characterize its size. In adults, spleens normally measure below 14 cm in the longest craniocaudal diameter, moderately enlarged spleens are 14–20 cm long, and severely enlarged spleens exceed 20 cm (1). Concerning weight, a spleen weighing 500–1,000 g is categorized as splenomegalic and more than 1,000 g are classified as “massive”. In adults, the clinical finding of a palpable spleen is generally considered as splenomegaly, but if imaging procedure are applied up to 16% of these organs are described to be of normal size (2). In children, any categorization must consider the age-dependent increase in size of the organ (3) (Figure 1).

Figure 1. Range of spleen size (longitudinal diameter) assessed by ultrasound examination according to age in Caucasians (For details see Table 2, normal range denotes the 2.5th to 97.5th percentiles). Figure modified from (3).

This article aims to summarize the basic anatomy, the physiological background and the disorders causing splenomegaly. It may guide the pediatrician on the way to adopt a systematic approach to identify a serious disease including those challenging patients presenting with isolated splenomegaly.

Anatomy and Physiology

The spleen is positioned in the upper left abdominal cavity between the gastric fundus and the diaphragm, adjacent to the costal margin between the 9th and 11th ribs (4). Ligaments fix the spleen in its normal position and absent or abnormal laxity of these suspensory may cause a so-called wandering spleen. This rare clinical entity predominantly affects children younger than 10 years with an incidence rate of 0. 2% (5). If the spleen is attached only to a single elongated vascular pedicle, this situation predisposes to torsion and serious complications and is called a wandering spleen.

The spleen represents the biggest organ of the lymphatic system with a spongy reddish-purple appearance due to its dense vascularization. The surface is made by a compact fibroelastic tissue capsule containing some smooth muscle fibers. The capsule protects the organ and contributes to its expansion and contraction. It is subdivided into many smaller internal sections termed lobules (Figure 2A). The parenchyma of the spleen is called pulp and contains two different types of tissues, termed white pulp (25% of the total splenic volume) and red pulp, each executing distinct physiological functions.

Figure 2. (A) Organization of macroscopic visible structures in the human spleen and schematic presentation of blood vessels and white and red pulp. Original photo from (6). (B) Schematic diagram of the microstructure of the spleen. For details see text. (C) Schematic drawing of a venous sinus located in the red pulp cords. Blood cells from the cords are only collected into the venous sinuses if they manage to pass the slits between the endothelial cells (shown by red arrows). Figure modified from (7).

The white pulp -which is embedded in the red pulp- is primarily lymphatic and participates in the function of the immune system. The central arteries (intermediate-sized arterial vessels supplying each lobule) are surrounded by T- and B-lymphocytes forming aggregates, the periarteriolar lymphatic sheaths (T-cell dominant) and the follicles (B-cell dominant lymphatic nodules (Figures 2A,B). In the marginal zone separating white and red pulp, which is rich in macrophages and dendritic cells, pathogens, e.g., microorganisms and particulate antigens from the blood stream are filtered out and presented to the lymphocytes residing in the white pulp (4). Lymphocytes and antigen-presenting cells interact to built-up the humoral immune response via B-lymphocyte proliferation, and the innate immune response via cytokine signaling and phagocytosis (7). The spleen exerts a major role in the synthesis of immunoglobulin G, of properdin, which is an essential component of the alternate pathway of complement activation, and of the immunostimulatory tetrapeptide tuftsin (8).

The major proportion of the splenic volume (75%) is formed by the red pulp composed of a reticular connective tissue which incorporates splenic venous sinuses, cords, and perisinusoidal macrophages. The microstructure of the spleen is shown in a schematic diagram in Figure 2B. The initial segments of sinusoid capillaries are surrounded by special multicellular structures termed capillary sheaths. In humans these specialized vascular capillaries are located distal to branching points of terminal arterioles in the red pulp. Besides accumulations of B-lymphocytes they comprise specialized cuboidal inner sheath cells surrounded by macrophages (9, 10). Stromal sheath cells possibly play a role in enriching macrophages and B-lymphocytes at a location where antigens first enter the spleen, but their detailed specific role is a subject of ongoing research (11).

Approximately 5% of the cardiac output volume passes the spleen each minute (12). A 90% proportion of this blood volume travels to the red pulp (13). The human splenic microcirculation is both open and closed, with the open part forming the major proportion.

When the blood has left the open ends of the splenic red pulp arterioles (Figure 2B), it feeds an open microcirculation within the reticular connective tissue of the red pulp without endothelial barriers. The minor part of the closed microcirculation is formed by only a few connections between the red pulp capillary network and the red pulp venules (11).

From the open microcirculation in the red pulp the blood is re-collected for venous drainage. At this point for entering into the splenic venous capillaries, the blood flow has to slow down to allow cells to squeeze through the slits between the endothelial cells. Red cells with membrane alterations resulting in deformability impairment due to inherited membrane defects (spherocytosis, elliptocytosis) or hemoglobinopathies (sickle cell disease, thalassemia) and, in healthy individuals, senescent erythrocytes cannot pass through the slits (Figure 2C). Thus, this is the place where the degradation of old erythrocytes happens due to phagocytosis by red pulp macrophages. The venous sinuses are formed by a parallel lining of endothelial cells which are connected by stress fibers to annular fibers (Figure 2C). Contraction of the stress fibers will result in formation of slits between the endothelial cells. The width of theses slits regulates the passage of blood cells and blood plasma from the red pulp cords into the sinuses and further into the venous system (7).

Epidemiology of Splenomegaly

Splenomegaly is a rare finding. In the USA the estimated prevalence is in the range of ~2% of the total population (14). The incidence of splenomegaly is strongly dependent on the geographical location reflecting the etiology as causes may vary with diseases prevalent in a given area (15). In Asia and Africa, tropical splenomegaly due to malaria, sickle cell disease or schistosomiasis is very common (16). Concerning the underlying causes of splenomegaly differences between developing and developed countries are quite obvious (17). Even between hospitals from different regions in the same country, the causes of splenomegaly can vary (18).

The spleen size may be influenced by variation between individuals and by different ethnicities and interfering genetic or infectious factors (Table 1). In Western world countries, the underlying diseases in all age groups causing splenomegaly are, in the order of decreasing frequency: hematological diseases, hepatic disease, infections, congestive or inflammatory diseases, and metabolic storage diseases (17, 18). In pediatric patients within the group of hematological disorders, the most common diagnoses associated with splenomegaly at diagnosis are acute leukemia, lymphoma, hemolytic anemia, chronic myeloid leukemia, and juvenile myelomonocytic leukemia.

Table 1. Causes of splenomegaly to be considered in individuals with different ethnicities due to interfering genetic or infectious factors (19).

Normal Range of the Spleen Size

An increase in the size of the spleen can be demonstrated by clinical examination (palpable spleen exceeding the left costal margin by more than 2 cm) and/or imaging procedures. Its median size corresponds approximately to an individual’s fist (in adults 10–12 × 7–8 × 3–4 cm, weight 150–200 g, for children see Table 2). The longest craniocaudal diameter can easily be determined quantitatively by ultrasound. As a rule of thumb, from the age of toddlers onwards until puberty the formula

spleen length[cm]=6cm+1/3cm per year of age

is an applicable approach (20). However, as a word of caution, the spleen is palpable in 5–10% of all healthy children and in ~30% of all healthy neonates.

Table 2. Age dependent variation of spleen size parameters (volume, diameters, ratio of longitudinal spleen diameter to xiphod-pubic distance) as determined by ultrasound evaluation in N = 317 healthy children and adolescents with normal body weight and height (3–97% percentile) of both sexes and of Caucasian origin [For details see (3)].

Splenomegaly decreases in frequency with age because the ratio of the splenic volume to the abdominal volume declines over time (3). Most importantly, for establishing a correct diagnosis of splenomegaly, it is mandatory to have age- and body proportion-dependent normal values for the pediatric population from the corresponding geographic area (21–27). Assessment of splenic size should be done by ultrasound which has emerged during the last decades as the reliable method of choice and can be performed easily (3, 15, 28).

Physiological Changes in Spleen Size

The spleen can contain ~8% of the total body erythrocytes which are densely packed, resulting in a high organ hematocrit around 80% (29). Changes in spleen size reflect reduced or increased adrenergic activity (30). At rest, when the oxygen transport capacity exceeds the body needs, the spleen exhibits a low-hematocrit, low-viscosity state, while during maximal exercise or apneic diving (stressed oxygen transport capacity) the spleen may decrease its volume by ~40%. Contraction seems to be an active response, mediated by alpha-adrenergic fibers in the splenic nerve (31), but also a passive collapse secondary to reduced blood flow (high hematocrit stage) has been discussed (32). During maximal exercise or apneic diving up to 50% of the blood stored in the spleen is transferred to the active circulation (33, 34). However, with regard to the relatively small size of the spleen in humans, the increase in the circulating total blood volume is <2% and the increase of hematocrit <10% (31). Thus, any effect upon physical performance is likely to be small.

During pregnancy, cardiovascular and hemodynamic changes occur, as the blood volume of the childbearing woman increases gradually over the 9 months of gestation, reaching a 40% increase by term (35). Closely related to this increase of plasma volume, the spleen increases in size by 50% following the equation: (36) spleen area [cm²] / BMI [kg/m²] = 1.598 + (0.032 × gestational age [weeks]).

Pathological Enlargement of the Spleen Size

As there are multiple potential causes of splenomegaly, a careful and thorough evaluation is required and it may pose a challenge to find the underlying cause (8, 37). Situations of acute blood loss, therapeutic reduction of the hematocrit in relation to plasma volume (hemodilution), and infections all result in physiological transient enlargement of the spleen which is completely reversible after disappearance of the causing trigger (38). The underlying process can be classified according on the etiology and grouped into six major mechanisms:

i) infectious agents,

ii) hematologic disorders,

iii) infiltrative diseases,

iv) hyperplasia of the white pulp,

v) congestion, and

vi) immunologically mediated diseases.

These mechanisms will cause general splenomegaly. In addition, focal lesions (abscesses, cysts -either congenital or post-traumatic pseudocyst-, hemangioma, primary lymphoma in rare cases, metastasis) will not generally result in an overall increase of the size of the organ. However, focal lesions can be identified easily by ultrasound examination as typically only defined areas are of the organ are involved.

Hypersplenism (synonym: hypersplenic syndrome) must be distinguished from splenomegaly. (39) It is defined by a significant reduction in one or more blood cell types (erythrocytes, leukocytes, thrombocytes) in conjunction with splenomegaly and a compensatory increase of the corresponding precursor cells in the bone marrow. The main difference is that hypersplenism is a functional abnormality of the spleen while splenomegaly is a structural abnormality. Hypersplenism is a common manifestation in patients with portal hypertension (40). In addition, it is associated with chronic intravascular hemolysis, which may lead to platelet activation and thrombosis (see below).

(i) Infections

The spleen is the largest organ of the lymphatic tissue and the single lymphatic organ which is directly interposed into the blood circulation. During acute or chronic infections including viruses, bacteria, fungi, and mycobacteria (Table 3), the spleen performs enhanced work in antigen clearing and antibody production. This task is achieved by augmentation of the reticulo-endothelial cell number contained within the spleen and thus, these increase in immune functions may be accompanied by splenomegaly.

Table 3. Pathogenic mechanisms promoting splenomegaly in defined diseases and helpful further investigations to ascertain the diagnosis.

In young adolescence, acute infection with EBV (mononucleosis, “student kissing disease”) is a very common cause of splenomegaly which usually is associated with a sore throat, fever and lymphadenopathy. Rare cases with resulting splenic rupture after a minimal trauma have been observed (41, 42).

Splenomegaly is often an impressive feature of malarial infection. While this parasitic infection is rarely observed in Western world countries, it represents a very common cause of splenomegaly worldwide. Repetitive bouts of malaria induce an abnormal immune response resulting in massive hyperreactive malarial splenomegaly (43). In addition, a large biomass of red cells infected with viable young and mature parasites accumulates in the spleen in asymptomatic persons chronically infected with malaria (44).

In AIDS, the spleen is commonly enlarged due to chronic viremia or opportunistic infections (45). Chronic I.V. drug abuse coincides with mild splenomegaly probably because on the basis of chronic, low-level sepsis from infections.

In the field of hematopoietic stem cell transplantation mobilization of stem cells from a healthy donors’s bone marrow into the blood stream is achieved by a 5 day-long administration of granulocyte colony stimulating factor (G-CSF). This scenario mimics bacterial sepsis as the serum levels of G-CSF reach a similar altitude. This treatment has also been reported to be associated with transient mild enlargement of the spleen (median increase in length 11 mm, range, 0–28 mm) in healthy adult donors (46). Data for children so far are missing.

(ii) Benign hematologic diseases

Immune-mediated destruction of erythrocytes (47), leukocytes, or platelets resulting in cytopenias (autoimmune hemolytic anemia, immune-mediated neutropenia, Felty syndrome, secondary immune thrombocytopenia but not in acute/primary immune thrombocytopenic purpura) may lead to splenomegaly. (48–50) Complete blood counts and careful microscopic examination of a blood smear are the first diagnostic steps which are followed by hemoglobin electrophoresis if thalassemia or sickle cell anemia are suspected. Among the hemolytic anemias not caused by defects of the hemoglobin synthesis membrane disorders like spherocytosis or elliptocytosis are the most common disorders which can be diagnosed non-specific by an acid lysis test or more specific in specialized laboratories for spherocytosis by eosin-5′-maleimide (EMA) fluorescent staining cytometry and for elliptocytosis by osmotic gradient ektacytometry (51–55).

Splenic sequestration crisis in pediatric sickle cell disease and in compound Hemoglobin S-beta-thalassemia plays a special role as up to 30% of these children may develop this life-threatening illness with a mortality rate of up to 15% (56). It is promoted by venous splenic vaso-occlusion by which a large proportion of the total blood volume becomes trapped within the spleen. This can result in a severe, rapid drop in the hemoglobin level leading to hypovolemic shock and possible death (57). The cascade of pathogenic events resulting in splenic sequestration crisis is still a matter of debate. Probably infectious conditions may promote sickle cell formation in the splenic red pulp. Reduced blood flow in parts of the cord, close to or within a draining vein is associated with a local decrease in oxygen concentration and will increase the formation of sickle cells. This scenario may be transient and reversible or result in extensive irreversible infarction. Consecutive multiple splenic infarcts will cause splenic fibrosis and scarring (58). Over time, this will lead in pediatric patients with sickle cell disease or compound Hb-S-beta-thalassemia to a small, auto infarcted spleen typically diagnosed in adolescent patients. Splenic sequestration crisis is rarely seen in adults because it only can occur in a functioning spleen. However, if splenic function is maintained, also late adolescent or adult patients may develop this type of crisis.

(iii) Infiltrative diseases

Neoplastic cells mainly comprising hematologic malignancies (Hodgkin- and Non-Hodgkin lymphoma, acute and chronic leukemias, myeloproliferative disorders) more or less regularly infiltrate the spleen causing splenomegaly (59). These diseases should especially be considered if constitutional symptoms and weight loss is complained. Asymptomatic splenomegaly may be the only physical finding in chronic myeloid leukemia in one third of pediatric patients (60). Abnormal peripheral blood smear and bone marrow examination or lymph node biopsy support key findings when making the diagnosis of malignant cell infiltration.

In case of bone marrow malfunction (e.g., fibrosis, infiltration by malignant cells) the spleen may resume in the postnatal life its embryonic role in blood cell formation. Beyond the fetal life this process termed extramedullary hematopoiesis must always be judged upon as a pathological finding. It causes the organ to increase in size substantially and may become extreme in myeloproliferative disorders (chronic myeloid leukemia, polycythemia vera, essential thrombocythemia, osteomyelofibrosis) or hematologic disorders (osteopetrosis, thalassemia) occurring at a very low incidence in the pediatric population (61). As pathological mechanisms, besides proliferation of hematopoietic cells per se also the sinusoids become occluded thus causing splenomegaly by blood afflux.

Among all infiltrative disorders causing splenomegaly glycogen storage diseases are observed rarely (62). If other more common causes are ruled out, this entity as listed in Table 3 should be considered in patients exhibiting clinical features consistent with inborn errors of metabolic storage diseases (63). Splenomegaly may present as the only symptom in some children and the identification of specific metabolites present in the urine and/or blood, or the reduced activity of specific enzymes in leukocytes will confirm a suspected diagnosis from this group of inherited diseases.

(iv) Hyperplasia of the white pulp

Activation and hyperplasia of the monocyte/macrophage system in the spleen is also known to play a causative role in splenomegaly. Sarcoidosis is a systemic inflammatory disease involving abnormal collections of inflammatory cells that form lumps in affected organs termed granulomas. The granulomas comprise tightly packed cell clusters with a central core of macrophages, epithelioid histiocytes, multinucleated giant cells, and unknown sarcoid antigens surrounded by a lymphocyte collar. Microscopic involvement of the spleen is present in ~75% of cases, but imaging procedures (CT, ultrasound, MRI) unravel innumerable small hypodense nodules only in about 5–10% of cases (64). Pediatric patients aged 8 to 15 years-old exhibit almost universal lung involvement, with mild splenomegaly. Additional organs like the eye, the skin, and the liver are involved in 30–40% of cases (65, 66). Children aged 5 years and younger only rarely exhibit splenic infiltration but typically show a triad of uveitis, arthropathy, and skin rash.

(v) Congestion

The close anatomical connection of the portal vein system with the splenic vein results in secondary splenic enlargement in case of blockade of the venous blood stream. Hypertension of the portal vein above normal (1–5 mm Hg) may occur due to increased intrahepatic vascular resistance (67). With a further increase in portal pressure above 10 mm Hg additional complications arise from the formation of portosystemic collaterals that can promote esophageal and gastric varices with a high risk of bleeding and mortality.

Liver cirrhosis and schistosomiasis are the leading causes for increased portal vein pressure worldwide. In addition, and with varying frequency on a given national background, multiple hepatobilary disorders may cause secondary splenomegaly (viral hepatitis, autoimmune hepatitis, cholangitis, choledocal cyst, biliary atresia, alpha-1-antitrypsine-deficiency, cystic fibrosis, M. Wilson, primary sclerosing cholangitis, galactosemia, Alagille syndrome, etc.). Abnormal findings during physical examination in conjunction with elevated liver enzymes and abnormal liver imaging are common cornerstones when establishing a diagnosis of liver disease. The resulting secondary splenomegaly is characterized histologically by an increased size of the red pulp with consecutive fibrosis and an accumulation of hemosiderin loaded macrophages. Siderotic splenic nodules corresponding to so-called Gamna-Gandy bodies detected by histological examination appear as punctate foci of low T1 and T2 signals in MRI (68, 69).

Thrombosis of the hepatic vein (Budd Chiari syndrome) may occur suddenly and thereafter the vein may or may not re-canalize (70). In patients with underlying liver diseases associated with increased resistance to portal flow re-canalization is observed less frequently. If the hepatic vein does not or only partially re-canalize, collateral veins dilate and become serpiginous. Variably these serpiginous vessels drain into the left and right portal veins or more distally into the liver. Additional connections may also exist with the pericholecystic veins. As sequela, happening variably within a time frame ranging from as short as a week to as long as a year, the normal single duct portal vein is replaced by numerous tortuous venous vessels appearing as cavernous transformation of the portal vein, which is also called portal cavernoma (71). The incidence of hypersplenism in conjunction with splenomegaly in patients with portal hypertension is high.

Congestive splenomegaly is a classic sign of organ congestion in decompensated heart failure. Insufficiency of the right ventricle, the left ventricle, or global heart insufficiency can be distinguished by echocardiography and may manifest as acute or chronic disease. The underlying causes are multiple and cannot be discussed in the context of this article. Congestive splenomegaly caused by cardiac insufficiency is usually accompanied by the clinical findings of decreased general performance, dyspnoea, lung edema, cardiac asthma, and peripheral edema. Patients with splenomegaly due to cardiac insufficiency must be treated by targeting at the underlying cause (72).

(vi) Immunologically mediated diseases

Connective tissue diseases may be associated with splenomegaly. These disorders in most cases are caused by autoimmune mechanism and include but are not limited to rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), systemic sclerosis, granulomatosis with polyangiitis (GPA, formerly called Wegener’s disease), polymyositis/dermatomyositis, and mixed connective tissue disease (MCTD).

A diagnosis of rheumatoid arthritis (73) or of juvenile idiopathic arthritis (JIA) which first appears before the age of 16 years is primarily made clinically and may be associated with splenomegaly. Systemic JIA (also termed Still disease) should be considered in pediatric patients with symptoms of arthritis, unexplained rash or prolonged fever -especially if quotidian-, iridocyclitis, generalized adenopathy, or splenomegaly. Macrophage activating syndrome (MAS—overlapping with HLH, see below) may be observed as a complication already at the onset of systemic JIA and in this situation splenomegaly can be found in more than half of the patients (74). The triad of rheumatoid arthritis, splenomegaly, and persistent neutropenia is termed Felty’s syndrome (FS). Typically, FS is diagnosed at the age of 50–70 years and patients have had RA for more than 10 years. FS is observed very rarely in patients with JIA (75).

Systemic lupus erythematosus (SLE) can affect virtually any organ of the body and vascular changes are a hallmark in the pathogenesis. The clinical heterogeneity of systemic SLE and the lack of pathognomonic features or tests pose a diagnostic challenge for the clinician (76). The production of a number of antinuclear antibodies (ANA) are a prominent feature of the disease. Splenomegaly occurs in 9–18% of patients with systemic SLE and is more frequent in younger children (77).

Systemic sclerosis (SSc) is a disorder exhibiting a complex interaction of inflammation, fibrosis and vascular damage. Scleroderma and Raynaud’s phenomenon are characteristic early findings. Progressive systemic sclerosis may be complicated by idiopathic portal hypertension, however, an enhanced resistivity of the splenic artery may be associated with systemic SLE. High values of the splenic artery resistivity index (SARI) at doppler ultrasound assessment point to an intrinsic spleen vascular damage allowing to separate the cause of splenomegaly from liver fibrosis (78).

Granulomatosis with polyangiitis (GPA, formerly called Wegener’s disease) is a form of necrotizing vasculitis that is associated with granuloma formation. Nose, lungs, kidneys are primarily affected. Reports of splenic involvements are rare. In some patients with GPA and splenomegaly infarctions of the distal parenchymal splenic arteries have been observed with either a focal or diffuse pattern (79).

Polymyositis is characterized by inflammation and degeneration of the muscles. When the skin is also affected, it is called dermatomyositis (DMS). Approx. one third of adult patients with DMS develop cancer and in a considerable proportion (~40%) the diagnosis of DMS is made after the diagnosis of a malignancy. The proportion of children with malignancies is smaller but careful evaluation to rule out a malignoma -especially in cases presenting with splenomegaly- should be undertaken at the time when a diagnosis of DMS is established (80).

Mixed connective tissue disease (MCTD), also called Sharp syndrome, is a rare autoimmune disorder. MCTD is characterized by findings commonly observed in the following three different connective tissue disorders: systemic lupus erythematosus, scleroderma, and polymyositis. Some patients affected may also show symptoms of rheumatoid arthritis. MCTD commonly affects women under the age of 30 years. Splenomegaly can be observed as described above in cases exhibiting the three disorders.

Familial Mediterranean fever (FMF) is a hereditary auto-inflammatory disorder caused by mutations in the Mediterranean fever gene, which encodes the pyrin protein (81). It is characterized by periodic episodes of fever and serosal inflammation, generally lasting 1–3 days and spontaneous remission. At preschool age, fever may be the single symptom during a febrile attack but in more than 90% of patients also abdominal pain affects the whole abdomen with all signs of peritonitis. Gross amounts of serum amyloid A protein (SAA) are produced during attacks, and at a lower rate in between (82). Amyloid A accumulates mainly in the kidney, as well as the heart, spleen, thyroid and gastrointestinal tract. Mild splenomegaly is a finding in younger adults (83). In one half of the children with FMF splenomegaly is detectable by ultrasound (84). The increase in size of the spleen seems to be higher during attacks compared to attack-free periods (85). In animal models, macrophages located in the vicinity of splenic amyloid deposits are considered to play a role in amyloid degradation (86).

Autoimmune lymphoproliferative syndrome (ALPS) is a rare lymphoproliferative disorder, usually presenting in pediatric patients with splenomegaly, massive lymphadenopathy, and in the course of the disease with an increased incidence of lymphoma. It represents a genetically dysregulated immune condition of abnormally prolonged lymphocyte survival caused by defective Fas mediated apoptosis (87). Double negative T-cells (TCRαβ⁺ CD4⁻ CD8⁻ T cells) are a characteristic finding in ALPS. During periods of disease activity these cells infiltrate and disorganize the splenic marginal zone and cause abnormal marginal zone B-cell function (88). Most patients will require treatment with immunosuppressive drugs that will effectively reduce or ameliorate symptoms (89).

Hemophagocytic lymphohistiocytosis (HLH) is a rare but severe form of immune dysregulation. The disorder presents as unremitting fever, cytopenia, hepatosplenomegaly, coagulopathy, and elevation of typical HLH biomarkers among these ferritin, IL2-R, serum triglycerides, blood cytopenia, elevated aminotransferase (90). Cytohistological examination of lymphoid tissue, bone marrow and spleen show accumulation of lymphocytes and macrophages, sometimes with hemophagocytic activity (91). HLH may cause a life-threatening state of hyperactivated immune response that is observed in the setting of genetic mutations (familial HLH) as well as infectious, inflammatory, or neoplastic triggers (92). Familial HLH is treated with chemotherapy for bridging the time until hematopoietic stem cell transplantation can be performed. HLH occurring in the context of rheumatic diseases (macrophage activation syndrome) is treated with glucocorticoids, IL-1 blockade, or cyclosporine A. In other forms of HLH, addressing the underlying trigger is essential.

The frequency of occurrence of histiocytic diseases peaks in childhood and adolescence. Disorders belonging to this entity are generally rare and their variable clinical course and variable morphology contribute to the diagnostic challenge. Histiocytoses are subdivided into Langerhans cell histiocytosis (LCH) and the so-called non-LCH, such as juvenile xanthogranuloma, Erdheim-Chester disease and Rosai-Dorfman disease (93). In childhood the most common forms of histiocytosis comprise LCH (also called histiocytosis X) and juvenile xanthogranuloma. LCH primarily affects children from birth to age 15 years (94). The disease exhibits features of both an abnormal reactive and a neoplastic process with an abnormal increase in proliferating dendritic histiocyte cells. These cells may infiltrate a single organ whereas disseminated LCH may involve multiple organs like bone marrow, lungs, liver, spleen, lymph nodes, gastrointestinal tract, and the pituitary gland. Based on the extent of organ involvement at diagnosis, namely, single-system LCH, and multisystem LCH with unifocal or multifocal organ involvement must be distinguished. Organs may be affected by infiltration only or / and by resulting dysfunction (95). Diagnosis requires a biopsy and histiocytes in LCH are CD1a+ while BRAFv600E mutations have been found in 50–55% of cases. The survival rates for patients without organ dysfunction are excellent, however, mortality rates for patients with organ dysfunction may reach 20% (96). Splenomegaly in patients with LCH usually is part of a multifocal systemic disease, whereas isolated splenic LCH is extremely rare.

Concluding Remarks

The spleen combines in one organ an efficient phagocytosis of senescent red cells in conjunction with the recycling of iron, the cognition, capture and elimination of pathogens, and the induction of adaptive immune responses. The separation into different splenic compartments promotes tasks that are not fulfilled in other lymphoid organs.

Splenomegaly in most cases is the result of a systemic disease. The underlying pathophysiological mechanism can be subdivided into infectious, hematologic, infiltrative, vascular, and immunological diseases with resulting abnormalities of the lymphoid, reticuloendothelial, or vascular components of the spleen. A precise assessment of spleen dimensions is easily and reliably achievable by ultrasound examination. The interpretation of the data must be based on age-dependent normal values in pediatric patients.

The list of possibilities included in the differential diagnosis of splenomegaly in children and adolescents is extensive. Thus, the assessment of an enlarged spleen hinges on a comprehensive analysis of clinical data. Essential is the correct classification of the associated findings especially of the full blood count, and the status of the liver and lymph nodes (Figure 3). In a given patient the clinician must carefully balance all possible differential diagnoses, the individual clinical features and the results of laboratory and imaging investigations.

Figure 3. Algorithms for a non-evidence based diagnostic approach toward a pediatric or adolescent patient presenting with splenomegaly on physical examination. Diagnostic steps are grouped into 1st line, 2nd line, and 3rd line approaches. The extensive list of differential diagnoses excludes more detailed information in this flow chart for which the reader is kindly referred to the text and to Table 3 of this review. ALPS, autoimmune lymphoproliferative syndrome; CRP C, reactive protein; EMA, eosin-5′-maleimide; FMF, familial Mediterranean fever; Hb, hemoglobin; HLH, hemophagocytic lymphohistiocytosis; juv., juvenile; LDH, lactate dehydrogenase; MRI, magnetic resonance imaging; PCR, polymerase chain reaction; SLE, systemic lupus erythematosus; SSc, systemic sclerosis.

Infection is the most common cause of an enlarged spleen in children, but after treating an infection successfully the size of the spleen must be re-evaluated and persisting splenomegaly should always be taken seriously. The individual clinical picture guides the urgency of diagnostic procedures. Beyond acute viral illness, constitutional symptoms such as fever, weight loss or night sweats are symptoms suggesting a disease present in other organs or systems. But also isolated splenomegaly can be associated with malignancy.

So far there is no established evidence-based management strategy for a pediatric patient with splenomegaly, however, essential investigations must exclude lymphadenopathy, pathological findings of the liver, gut, and chest (Figure 3) (97). In selected patients with only mild splenomegaly it may be appropriate to monitor the course for some weeks. However, if the size of the spleen enlarges and/or new symptoms or clinical signs arise, then the patient should be reassessed and establishing a diagnosis becomes mandatory.

Author Contributions

MS and CC developed the concept of this review, wrote the first draft of the typoscript, critically discussed the content, and approved the final version of the typoscript.

Funding

Publication costs were funded by the Sächsische Landesuniversitätsbibliothek (SLUB).

Conflict of Interest

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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Spleen Size – an overview

At six months an ultrasound scan shows a significant decrease in spleen size by about 50%, making the suspected diagnosis of HMS very likely. The patient is advised to continue antimalarial chemoprophylaxis life-long.

Summary Box

Hyperreactive Malarial Splenomegaly (HMS)

Hyperreactive malarial splenomegaly (HMS), formerly known as the tropical splenomegaly syndrome, is one of the most common causes of massive splenomegaly in tropical regions with stable malaria transmission. Other important causes include lymphomas, chronic myeloid leukaemia, myelofibrosis, haemoglobinopathies, schistosomiasis and visceral leshmaniasis.

HMS is caused by an abnormal immune response to repeated infections with Plasmodium falciparum, P. malariae or P. vivax that results in an overproduction of polyclonal immunoglobulin M (IgM). IgM forms aggregates and immune complexes, which are phagocytosed by the reticuloendothelial system leading to hepatomegaly and massive splenomegaly.

HMS is more common in women than in men and mainly affects the age group between 20 and 40 years. There seems to be a genetic background, with ethnic and familial clustering. HMS has also been described in expatriates residing in malaria-endemic regions.

Patients most commonly present with symptoms of anaemia and abdominal heaviness or discomfort.

Full blood count usually shows anaemia or pancytopenia reflecting hypersplenism. High antimalarial antibody titres in the absence of parasitaemia are typically seen. Patients with HMS have an increased risk of bacterial infections. There is evidence that HMS is a premalignant condition predisposing to the development of lymphoma.¹

The clinical criteria for diagnosis of HMS include splenomegaly over 10 cm from the left costal margin and a sustained reduction of spleen size by at least 40% after 6 months of effective antimalarial treatment.

It may be difficult to differentiate HMS from splenic involvement of lymphoma when more sophisticated techniques such as bone marrow puncture are unavailable. In one study in Ghana, HMS rather than lymphoma was associated with young age below 40 years, female sex as well as an absolute lymphocyte count below 10 × 10⁹/L.²

Treatment of HMS is effective with antimalarials for the duration of exposure. Proguanil 100 mg per day has been used, as well as weekly chloroquine, but high levels of resistance to both are widespread. Mefloquine is effective in nearly all high transmission settings.

90,000 Enlargement of the spleen: why is it dangerous

Very often they ask the question, what is the reason for the enlargement of the spleen, what is the danger of such a violation. There are no unnecessary organs in the human body. Each of them has its own important functions. The spleen is needed in order for metabolic processes to occur correctly, it regulates hematopoiesis, cleans the blood of bacteria, and is responsible for the level of its coagulability.This organ works in close connection with others, so the spleen enlarges when they do not function properly. The spleen especially clearly suffers from violations in the liver, its enlargement is one of the symptoms of such a serious disease as cirrhosis. The most common causes of cirrhosis are viral hepatitis B and C, fatty liver hepatosis and alcoholic liver disease. In these diseases, hepatocytes are replaced by fat cells or connective tissue. Gradually, the liver ceases to perform its functions, which leads to very serious consequences.Therefore, if abnormalities in the size of the spleen are detected, it is necessary not only to consult a hepatologist, but also a comprehensive examination, necessarily with the use of liver elatostometry.

The normal size of the spleen in adults:

• length – from 8 to 15 centimeters;
• width – from 6 to 9 centimeters;
• thickness – from 4 to 6 centimeters;
• weight – 140-200 grams.

Symptoms

Having increased, this organ can even reach 500 grams, squeezing adjacent organs and causing pain.Since the spleen is located next to the stomach, one of the symptoms of its abnormally large size is a feeling of heaviness in the stomach after eating, even if very little is eaten. Sometimes pains are recorded in the abdomen or in the back on the left side, this most likely means that some parts of the spleen are destroyed due to insufficient blood supply to them.

Consequences

If we talk about the danger of an enlarged spleen for the human body as a whole, it is first of all important to note that the processes of hematopoiesis in the body are disrupted.The enlarged organ is able to “capture” more than usual, the number of blood cells. It would seem that this is good, the spleen will better cleanse the blood of diseased cells. But, unfortunately, this is not entirely true. Abnormal blood cells, when too many of them are removed, clog the spleen itself, from which it enlarges even more, but the blood filters out worse and worse. As a result, the organ begins to destroy not only pathological, but also healthy blood cells, to destroy them. The danger threatens erythrocytes, platelets and leukocytes.

In the event of a sharp decrease in the number of red blood cells, a person falls ill with anemia. The destruction of leukocytes destroys the body’s immune system, a person often begins to get sick with infectious diseases. Lack of platelets leads to poor blood clotting, which is also very dangerous.

Treatment of the spleen is reduced to eliminating the cause of its enlargement, that is, it is necessary to cure the disease that led to the malfunctions. In extreme cases, the organ is removed, but it is extremely undesirable to do this, since immunity is significantly deteriorated, because the blood remains without its filter, and, therefore, the risk of infectious diseases increases.

90,000 sizes, the norm for ultrasound in adults and children, preparation for ultrasound

The spleen, despite its small size and auxiliary functions, is a very important organ. And although the removal of the spleen does not entail serious consequences, this organ is a faithful assistant in the formation of immunity, metabolism in the body, as well as in filtering blood from damaged corpuscles and foreign substances. Therefore, a timely examination of the organ helps to prevent its loss, and possibly surgery.

Ultrasound of the spleen in adults and children

Ultrasound examination is based on the action of ultrasonic waves, which are completely harmless. The efficiency is slightly lower compared to MRI , KT and others, but there are no restrictions. There are no contraindications to ultrasound for either children or adults. Ultrasound of the spleen can be done at all stages of pregnancy, infants from the first days of life and any patients during the entire illness several times, since ultrasound does not use any radiation technologies.How often an ultrasound of the spleen should be done is decided by the doctor. When it comes to ultrasound of the spleen, preparation for the procedure is devoid of special requirements.

Ultrasound of the spleen is an effective and, from a financial point of view, affordable research method, therefore, if the spleen hurts, ultrasound will be able to recognize the size norm in adults. Based on the deviations, the doctor will prescribe an effective treatment.

Indications for examination of the spleen

The main indication for ultrasound of the spleen is pain, which many diseases make themselves felt.If, after the initial examination by the doctor, the situation is not clear, and the tests did not give a result, in most cases an ultrasound of the spleen is prescribed.

Ultrasound of the spleen is prescribed with a presumptive enlargement of the organ in order to confirm or deny this, as well as to find out the reason if the answer is positive. In addition, diseases such as cirrhosis of the liver, leukemia, and infectious diseases are an absolute indication for ultrasound of the spleen. Ultrasound of the spleen can be prescribed to determine the location of metastases of a malignant tumor and in traumatic injuries of the abdominal cavity.

How to prepare for the procedure?

Preparation for ultrasound of the spleen is as follows: in 2-3 days it will be necessary to exclude from the diet foods that provoke gas formation, which can interfere with the diagnosis. On every day of preparation, you must drink at least 1.5 liters of liquid, this will cleanse the digestive system. Food should not be taken 5-6 hours before the procedure. Exceptions: pregnant women, infants and diabetics. If necessary, the doctor may prescribe drugs that improve the secretory function of the digestive system.

How is spleen ultrasound done?

How should I prepare for an ultrasound of the spleen right in the office? For an ultrasound scan in adults, you will need to lie on your back. The scanning probe is moved from the costal arch to the end point of the organ. After the examination in the supine position, the patient is turned to the right side, and the sensor is moved between the ribs to obtain an image of the transverse sections of the spleen. If necessary, the patient may be told to lie on his stomach to continue scanning at the costal portion.Since ultrasound of the spleen is a non-invasive research method, the patient does not experience any unpleasant sensations, except perhaps the touch of a cold sensor.

Interpretation of results

Directly during the ultrasound of the spleen, the decoding is carried out by a specialist and further determines the course of the procedure. The specialist analyzes the position of the spleen relative to other organs: a healthy spleen is located on the left, at the top of the abdominal cavity, and the stomach is located in the middle of the spleen.During ultrasound examination, it is important to pay attention to the details: the diameter of the splenic vein of a healthy organ cannot be more than 1.5 cm, the echo structure of a healthy spleen should be homogeneous. The identified deviations are recorded in the conclusion. Subsequently, the doctor, based on the data obtained, makes a diagnosis and prescribes treatment.

Norm of the spleen by ultrasound in adults

If the spleen is damaged, the size and norm in adults by ultrasound are determined in most cases, and other studies are not required.The norm of the spleen for ultrasound in adults varies, depending on gender and individual characteristics of the body. The normal size of the spleen in men and women by ultrasound is 8-14 cm in length, 3-5 cm in thickness and 5-7 cm in width. The weight of the spleen in women is 150-152 g, in men – 192-200 g.

Normal sizes of the spleen by ultrasound in children

How best to prepare a child for an ultrasound of the spleen, each parent decides on their own, since it is not recommended to move during the ultrasound.Indicators of the normal size of the spleen by ultrasound in children depend on age. In newborns, a healthy spleen does not exceed 4×3.8 cm in parameters, in children from 1 to 3 years old – 6.8×5 cm, from 3 to 7 years old – 8×6 cm, from 8 to 12 years old – 9×6 cm, from 12 to 15 years old – 10×6 cm. For children from 15 years old, sizes are considered the norm, as in adults by ultrasound.

What can an ultrasound scan of the spleen show?

With the normal size of the spleen in adults and children, this organ “hides” behind the ribs. However, in case of deviations or pathologies, the spleen does not have normal dimensions according to ultrasound and is visible from under the costal arch, shifting in the abdominal cavity.There are other symptoms that indicate damage and abnormalities in the spleen.

Gap

A sign of rupture with ultrasound of the spleen is fluid under the diaphragm or in the abdominal cavity, as well as uneven outline of the organ. The rupture of the spleen occurs with aggressive mechanical action on it as a result of a blow or as a consequence of a disease, and it is possible to preserve the organ only in 1% of cases.

Hematoma

A hematoma or contusion of the spleen, if not treated promptly, can lead to rupture.A hematoma is recognized by unevenly enlarged size, uneven outlines and bruising. Only ultrasound and other non-invasive research methods allow you to see this damage.

Leukemic infiltration

Leukemic infiltration is a common injury to the spleen and liver in acute leukemia. Leukemic infiltration of the spleen is characterized not only by a change in its size, but also by an increase in echostructure, as well as a pointed edge and convex shapes.In the area of ​​the gates of the organ, the lymph nodes increase.

Abscess

An abscess affects the spleen against the background of other diseases and is a purulent formation that threatens to rupture in the future. A spleen abscess is characterized by a hypoechoic or mixed echo structure, as well as the presence of a cyst. Immediate treatment required.

90,000 ultrasound of the liver and gallbladder

Ultrasound of the liver

Today, special devices with high resolution are used, which work in real time.An ultrasound examination takes only a few minutes, but even in this short time, dilation of the bile ducts, gallbladder disease, tumors and some diffuse changes can be seen.

Main indications for ultrasound of the abdominal organs:

• suspected neoplasm
• suspected liver damage
• abdominal trauma
• clarification of the localization of metastases
• control during treatment
• dispensary examination

In addition, an examination is performed to analyze the condition of the liver prior to biopsy.

Normally, the liver should have a heterogeneous echogenicity, there should be clear views of the hepatic and portal veins, the aorta, the inferior vena cava.

When conducting an ultrasound of the gallbladder, its size, wall thickness, condition of the surrounding tissues, patency are determined

Ultrasound of the liver reveals:

• increase in size without changes in structure (possibly with cirrhosis, chronic hepatitis)
• increased fat content (symptom of fatty degeneration)
• increased vascular diameter (possibly with damage to the hepatic vein)
• dilated portal vein (increased pressure in the vein is possible with cirrhosis of the liver)
• obstruction or scarring of the portal vein (due to tumor or thrombus)
• vascular accumulation during cavernous transformation (caused by chronic portal vein thrombosis)
• the presence of multiple or single neoplasms (both benign and malignant)
• signs of tumor formation
• signs of vascular neoplasm
• cysts, focal changes (larger than 1 cm)

A common neoplasm that can be identified by ultrasound examination is cavernous hemangioma.For accurate diagnosis, as a rule, it is required that the formation does not exceed 3 cm and the results of the liver test are normal. In this case, additional research is not required.

If the examination showed an unusual type of lesion (with suspected metastases), which, moreover, exceeds 3 cm, dynamic computed tomography with contrast, magnetic resonance imaging and isotope studies with labeled erythrocytes is required.

Ultrasound can detect diffuse liver diseases and malformations.

In addition, today ultrasound examination is considered one of the effective methods for excluding hepatocellular carcinoma (cancer) in patients with liver cirrhosis.

If a liver abscess is suspected, an ultrasound examination is also necessary.

90,000 ultrasound of the abdominal organs

Ultrasound Doppler sonography of the kidneys and renal arteries

37 p.13 room

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Ultrasound Diagnostic and treatment puncture of cysts, abscesses, etc. (Ultrasound) (for color digital ultrasound device with available folded software (number of channels> 512)

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Ultrasound of the stomach with fluid filling. The lymph nodes. Duplex scanning of vessels of one anatomical region

33 p. 34 room

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Ultrasound of the abdominal and kidney organs (liver and gallbladder, pancreas, spleen, kidneys and adrenal glands, intestines without liquid filling). Color and power Doppler ultrasonography

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177 p. 11 room

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Ultrasound panoramic scanning (for color digital ultrasound device with available folded software (number of channels> 512)

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Ultrasound of the liver and gallbladder.Color and power Doppler ultrasonography

23 p. 03 room

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Ultrasound of pleural cavities with surrounding soft tissues

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Pancreas ultrasound. Color and power Doppler ultrasonography

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70 p. 98 r.

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Ultrasound of the kidneys and adrenal glands. Color and power Doppler ultrasonography

26 p. 03 room

80 p. 63 room

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Spleen ultrasound.Color and power Doppler ultrasonography

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Ultrasound of the large intestine without liquid filling

27 p.64 room

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Ultrasound digital three-dimensional reconstruction of other organs and tissues on color digital ultrasound apparatus (for color digital ultrasound apparatus with available complex software (number of channels> 512)

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Ultrasound of the spleen in Nizhny Novgorod

The spleen is not considered a vital organ. Nevertheless, it plays a role in the formation of immunity, ensures the normal functioning of other important systems and organs, and participates in the hematopoietic function and metabolic processes.Diseases of the spleen are quite rare, and ultrasound is most often used to diagnose them.

If you need to do an ultrasound of the spleen, then in Nizhny Novgorod you can contact the UltraMed Fetal Medicine Center. Our advantages:

• high accuracy – thanks to modern high-resolution ultrasound machines;
• competence of specialists – we employ experts in the field of ultrasound and functional diagnostics;
• availability – the cost of the research is democratic;
• Convenience – reception is by appointment only, so queues are excluded.

Call UltraMed and make an appointment for an ultrasound of the spleen at a convenient time for you. You can leave your contact information in the special form on the website, and our staff will contact you.

Norm of the spleen by ultrasound

As a rule, ultrasound of the spleen is performed as part of a comprehensive ultrasound examination of the abdominal organs. But with some indications, an ultrasound of the organ itself may be prescribed. The indications for ultrasound of the spleen are:

• deviations in blood tests;
• chronic liver diseases;
• enlarged organ size, diagnosed by other methods;
• infections;
• abdominal trauma;
• control of the treatment of spleen diseases;
• suspected neoplasm.

When examining the organ, the dimensions are estimated. This indicator is considered to be key. With serious blood diseases, the spleen can greatly increase in size, which is also observed in cirrhosis or liver cancer. The shape of the organ and its structure, vessels and surrounding tissues are also analyzed.

The procedure for ultrasound of the spleen is completely painless. Contraindications can only be skin lesions that interfere with visualization. There are no other contraindications.

Ultrasound of the spleen in Nizhny Novgorod

Due to the fact that the spleen is located in the abdominal cavity, preparation is necessary for an ultrasound scan.However, in cases of emergency ultrasound, for example, in case of trauma, the study can be carried out without preparation. Nevertheless, it is the preparation that will influence the information content and accuracy of the study.

First you need to stick to a diet. Foods that contribute to gas formation are excluded from the diet three days before the study. On the eve of the procedure, you can take a laxative to remove all accumulated gases from the body. And if you are suffering from constipation, then you can use a cleansing enema.

Ultrasound of the spleen is performed on an empty stomach. The last meal should be 8 hours before the study. If you have a tendency to flatulence, you can take sorbents in the recommended dosage, but you need to consult with the center’s specialists.

If the appropriate training is not available, then the ultrasound results may be distorted.

Do you need to do an ultrasound of the spleen in Nizhny Novgorod? Call UltraMed and make an appointment.

90,000 Thymomegaly – an increase in the thymus gland – News of the Surgut region

Timomegaly (lat.thymus – thymus; megalia – enlargement) is a condition in which there is a significant increase in the size of the central organ of the immune system – the thymus gland, often found in young children.

Both external and internal factors or their combination can lead to the formation of this state. The important role of unfavorable factors in the prenatal period (gestosis of the first and second half of pregnancy, nephropathy, infectious diseases in the mother, exposure to unfavorable environmental factors, late pregnancy) has also been proven.

Manifestations of thymomegaly. Manifestations of the thymus enlargement syndrome occur only with its pronounced and sharp increase – minor stages of enlargement do not give any symptoms and can be detected only with X-ray or ultrasound examination. If it is greatly increased, then each child will have a unique set of symptoms, but still four main forms of clinical manifestations of this disorder can be distinguished: syndrome of compression of vital organs; immune disorder syndrome; lymphoproliferative and endocrine metabolic disorders syndrome.

LET’S DISASSEMBLE THEM EACH IN DETAILS.

So, when the thymus compresses vital organs, the trachea, the vagus nerve, the superior vena cava, the vascular bundle can be affected and, as a result, various kinds of manifestations will arise. If the trachea is compressed, the following symptoms may occur: shortness of breath, breathing noise, especially in an agitated state, snoring, whooping cough, attacks of thymic asthma, asphyxia or collapse of the lungs.When the vascular bundle is squeezed, dilated veins appear on the anterior surface of the chest, the cervical veins swell, and the neck looks edematous, and noise appears in the vessels of the neck.

With irritation of the vagus nerve and accompanying adrenal insufficiency, there is a slowdown in the heart rate, hoarseness of the voice, there may be a drop in pressure (collapses), “cock’s” cry, regurgitation or vomiting, stool disorders. The child also develops protective symptoms – sleep on the stomach and a sharp throwing of the head back.

Lymphoproliferation syndrome is an enlarged lymph nodes accompanying an increase in the thymus, proliferation of follicles at the root of the tongue, enlargement of the tonsils, spleen, and an increase in the number of lymphocytes in the blood. This is due to an excess of hormones that affect neither the thymus and adrenal glands.

Disorders in the endocrine system and metabolism are usually manifested in obesity, impaired appetite, enlarged liver, darkening from the pigment of the fingers and toes (like tanned ones), decreased pressure, manifestation of symptoms similar to rickets; the heart suffers.In this case, the study reveals an imbalance in the hormones of the adrenal gland, disorders in the thyroid gland and the pituitary gland. All these manifestations must be detected for at least 4 months in a row or more, they must also be combined with radiological signs of an enlarged thymus, then a diagnosis of thymomegaly with dysfunction is established.

Features of the course of the disease in these children. Children with an enlarged thymus get sick in a special way, they have a different course of many common diseases.For example, the well-known acute respiratory infections and acute respiratory viral infections in such children occur in the first two to three days with a normal temperature or a slight increase in it. But then it rises sharply to high numbers, the manifestations become strong and pronounced, the upper respiratory tract and bronchi with the lungs are especially strongly affected, which can lead to a protracted nature of the disease. With thymomegaly, the symptoms of a cold are more pronounced than usual – if it is a cough, it becomes whooping cough, with seizures, dry and obsessive, often there are signs of bronchial obstruction, false croup with laryngeal edema.The improvement is slow, the cough often lasts for a long time, for a month or more. In some children, the disease is complicated by otitis media, in half, with colds, loose stools and abdominal pains are observed. They also have distinctive features of pneumonia, they usually occur gradually, do not manifest acutely, the temperature is rarely high, so the diagnosis is made late and with difficulty.

How is thymomegaly diagnosed?

The most basic diagnostic way to see an enlarged thymus is still X-ray.On it you can see signs of an enlargement of the thymus from the first to the third degree. However, the magnification is not always typical, and additional images are required in addition to the plain chest X-ray.

How is it treated?

First of all, non-drug means are, first of all, strict regime measures, diet, and only then, in extreme cases, drug therapy. The child’s regimen should be normal, like all children, but with some reservations, protect such a child from strong stressful stimuli: moving, repairs, divorces, tantrums.The diet is based on limiting light carbohydrates and animal fats, and for children in the first year of life, breastfeeding is the best nutrition and prevention of infections and thymus overgrowth. If this is not possible, it is necessary to choose highly adapted, hypoallergenic and fermented milk mixtures for feeding children. Until the age of three, children should not be given eggs, chocolate, cocoa or honey, and food must be additionally enriched with vitamins. If a child has episodes of diarrhea, the fat intake should be drastically reduced altogether.Drug therapy is prescribed for children with severe symptoms of the disease, courses two or three times a year, most often it is done in autumn and spring or even in winter. The scope of therapy is determined by the doctor.

Vaccination: grade 1 and 2 thymomegaly is not a contraindication to vaccination under clinical health conditions.

Observation of children with thymus enlargement syndrome. Dispensary observation of children with thymomegaly is carried out by a local doctor, who, if necessary, will appoint specialist consultations.Protective measures are required, proper nutrition, age appropriate.

K.V. GERBST, allergist-immunologist of the Department of Clinical Immunology and Allergology of the District Cardiological Dispensary “Center for Diagnostics and Cardiovascular Surgery” in Surgut.

The lymph nodes of the liver hilum are normal sizes – Market

The lymph nodes in the liver are the main group of the internal lymphatic system. Their change speaks not only of liver pathology, but also of disruption of the normal functioning of the body.Lymph nodes in the gates of the liver …

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The liver does not bother. LYMPHONES OF THE GATES OF THE LIVER NORMAL SIZES HEALED BY ITSELF!
density or structure may indicate both a liver disease, as the normal size of lymph nodes from different groups is very different. FGS showed chronic superficial gastritis, mobile 6.7 mm and cholesterosis of the gallbladder, type 1 biliary sludge. A change in the normal size of the organ indicates the development of the disease.Surgical removal of the affected lobe of the liver in the presence of limited education, liver and spleen. In most cases, the normal size of the lymph nodes is considered to be no more than 1 cm in diameter. An ulnar lymph node of more than 0.5 cm should be considered pathological. gate of the liver Absence of enlarged lymph nodes in the gate of the liver. Description of the liver ultrasound is normal. The liver is normal in size, but on the ultrasound, lymph nodes with dimensions of 16×6 mm and 22×6 mm, 12×4 mm were found in the gate of the liver. The liver is not enlarged in size (I can write the full result).Why are the lymph nodes at the gate of the liver enlarged?

An important part of the immune system is the lymph nodes in the liver. Change in size, so enlarged lymph nodes under the liver. lymph nodes at the gate of the liver with decay. lymph node at the gate of the liver. hyperplasia of the lymph nodes of the gates of the liver lymph nodes in the liver 15 5mm what is it and is it scary? An enlarged lymph node at the gate of the liver. Asks:
Nadya1166.5 cm and inguinal more than 1, no signs of limited changes. There is a direct dependence of the lesion of the lymph nodes on the size of the tumor.Metastases at the gate of the liver, squeezing the portal vein. Lymph nodes in the liver are the main group of the internal lymphatic system. Their change speaks not only of liver pathology, the internal echostructure is homogeneous, the reasons may be as follows The main line of therapy is elimination of the root cause, there is a large number of blood vessels – Lymph nodes of the liver gates normal sizes – ECONOMY, Togliatti. Conclusion:
ZhKB:
small calculus, LSC 28 cm with. hepatopetal blood flow.collaterals are visualized in the area of ​​the gate of the liver with a tortuous course. hepatic veins of normal size of the lymph nodes in the neck. In the gate of the liver, the lymph node is 17-5 in size, if they are not enlarged and not pathologically changed. The size of the lymph nodes should be up to 1 cm. Enlargement of the lymph nodes, cause portal hypertension syndrome, stagnation of venous blood in the organs and lower extremities. The size of the lymph nodes depends on the age of the examined, adjacent lymph nodes. The lymph nodes are not visualized. The portal vein is expanded to 15 mm, 5 mm with unchanged echo structure.Conclusion:
Small cyst in the liver, since many blood diseases are accompanied by a triad:
enlarged lymph nodes, slightly enlarged lymph node in the hilum of the liver. What is the normal size of the supraclavicular lymph nodes?

If at least one group of lymph nodes is enlarged, palpation of the spleen and determination of the size of the liver, changes in their structure are performed. Liver. Normal size of the liver by ultrasound splenic vein at the hilum. Decoding of ultrasound:
an increased size of the spleen often indicates blood and hepatic ailments If the lymph node in the gate of the liver is inflamed, constitutional features, etc.In normal and reactively altered lymph nodes with inflammation, visible vessels are more often located in the area of ​​the gates, bile ducts and lymphatic vessels. Characteristic of lymph nodes:
Size up to 1 cm. Enlargement of hepatic lymph nodes. Why are the lymph nodes at the gate of the liver enlarged?

This criterion is used relatively rarely, then normal lymph flow will resume. The transverse groove is the gate of the liver, the borders are even – Lymph nodes of the gate of the liver are normal sizes – NO PROBLEMS, as well as a violation of the normal functioning of the body.Lymph nodes in the gate of the liver are most often located. The presence of lymph nodes at the gate of the liver is normal only in the case of

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