What is the treatment for polycythemia vera? Explore the various therapies, including phlebotomy, medications, radiation, and symptomatic relief, to control this condition and reduce the risk of complications.

Understanding Polycythemia Vera and Its Treatment Objectives

Polycythemia vera (PV) is a rare blood disorder characterized by an overproduction of red blood cells, which can lead to a thickening of the blood and an increased risk of blood clots, heart attacks, and strokes. While there is no cure for PV, a range of treatments are available to help manage the condition and its complications.

The primary goals of PV treatment are to control symptoms, reduce the risk of serious complications, and bring the patient’s blood count back to a more normal level. This is typically achieved by reducing the number of red blood cells and the level of hemoglobin (the oxygen-carrying protein) in the blood, which helps improve blood flow and oxygen delivery throughout the body.

Phlebotomy: Removing Excess Red Blood Cells

One of the most common and effective treatments for PV is phlebotomy, a procedure that involves removing a pint of blood from the patient’s vein on a regular basis, typically once a week. This helps to lower the red blood cell count and bring the blood thickness closer to normal, reducing the risk of blood clots and other complications.

The process of phlebotomy is similar to blood donation, with the blood being collected in a sterile container. Patients may need to undergo phlebotomy treatments every few months to maintain their blood count within a healthy range.

Medications to Suppress Bone Marrow Activity

In addition to phlebotomy, your doctor may prescribe medications to help control the overproduction of red blood cells. Two common examples are:

1. Hydroxyurea: A cancer drug that can reduce the number of red blood cells and platelets in the blood, improving blood flow and thickness.
2. Interferon-alpha: A substance naturally produced by the body that can prompt the immune system to fight overactive bone marrow cells, helping to lower the red blood cell count.

Radiation Therapy to Suppress Bone Marrow

In some cases, radiation therapy may be used to help suppress the overactive bone marrow cells responsible for the excess red blood cell production in PV. This can help lower the red blood cell count and improve blood flow, but it also carries a risk of potentially developing other blood diseases, such as leukemia, in the future.

Treatments to Manage Symptoms and Complications

In addition to the primary treatments to control the underlying condition, your doctor may also prescribe medications or recommend other therapies to help manage the symptoms and complications associated with PV, such as:

• Aspirin: To relieve bone pain, burning sensations in the hands or feet, and reduce the risk of blood clots.
• Anti-itch medications: To help alleviate the itching that can be a common symptom of PV.
• Ultraviolet light therapy: To help relieve itching in some patients.

Emerging and Experimental Treatments

Researchers are continuously exploring new and innovative treatments for PV, including:

• Selective serotonin reuptake inhibitors (SSRIs): Low doses of these antidepressant medications have shown promise in clinical trials for reducing itching in PV patients.
• Imatinib mesylate: A leukemia drug that has been found to help reduce the need for phlebotomy and the size of enlarged spleens in some PV patients.
• JAK2 inhibitors: Drugs that target the abnormal JAK2 gene, which is the primary genetic driver of PV, are being investigated as potential new treatments.

As research continues, the treatment options for PV may continue to evolve and expand, providing patients with even more effective ways to manage their condition and reduce the risk of serious complications.

Working Closely with Your Healthcare Team

Effective management of polycythemia vera requires a collaborative approach between the patient and their healthcare team, which may include hematologists, oncologists, and other specialists. Regular monitoring, adherence to prescribed treatments, and open communication are crucial for ensuring the best possible outcomes for PV patients.

How is Polycythemia Vera Treated?

Polycythemia vera (PV) doesn’t have a cure. However, treatments can help control the disease and its complications. PV is treated with procedures, medicines, and other methods. You may need one or more treatments to manage the disease.

Goals of Treatment

The goals of treating PV are to control symptoms and reduce the risk of complications, especially heart attack and stroke. To do this, PV treatments reduce the number of red blood cells and the level of hemoglobin (an iron-rich protein) in the blood. This brings the thickness of your blood closer to normal.

Blood with normal thickness flows better through the blood vessels. This reduces the chance that blood clots will form and cause a heart attack or stroke.

Blood with normal thickness also ensures that your body gets enough oxygen. This can help reduce some of the signs and symptoms of PV, such as headaches, vision problems, and itching.

Studies show that treating PV greatly improves your chances of living longer.

The goal of treating secondary polycythemia is to control its underlying cause, if possible. For example, if the cause is carbon monoxide exposure, the goal is to find the source of the carbon monoxide and fix or remove it.

Treatments To Lower Red Blood Cell Levels

Phlebotomy

Phlebotomy (fle-BOT-o-me) is a procedure that removes some blood from your body. For this procedure, a needle is inserted into one of your veins. Blood from the vein flows through an airtight tube into a sterile container or bag. The process is similar to the process of donating blood.

Phlebotomy reduces your red blood cell count and starts to bring your blood thickness closer to normal.

Typically, a pint (1 unit) of blood is removed each week until your hematocrit level approaches normal. (Hematocrit is the measure of how much space red blood cells take up in your blood.)

You may need to have phlebotomy done every few months.

Medicines

Your doctor may prescribe medicines to keep your bone marrow from making too many red blood cells. Examples of these medicines include hydroxyurea and interferon-alpha.

Hydroxyurea is a medicine generally used to treat cancer. This medicine can reduce the number of red blood cells and platelets in your blood. As a result, this medicine helps improve your blood flow and bring the thickness of your blood closer to normal.

Interferon-alpha is a substance that your body normally makes. It also can be used to treat PV. Interferon-alpha can prompt your immune system to fight overactive bone marrow cells. This helps lower your red blood cell count and keep your blood flow and blood thickness closer to normal.

Radiation Treatment

Radiation treatment can help suppress overactive bone marrow cells. This helps lower your red blood cell count and keep your blood flow and blood thickness closer to normal.

However, radiation treatment can raise your risk of leukemia (blood cancer) and other blood diseases.

Treatments for Symptoms

Aspirin can relieve bone pain and burning feelings in your hands or feet that you may have as a result of PV. Aspirin also thins your blood, so it reduces the risk of blood clots.

Aspirin can have side effects, including bleeding in the stomach and intestines. For this reason, take aspirin only as your doctor recommends.

If your PV causes itching, your doctor may prescribe medicines to ease the discomfort. Your doctor also may prescribe ultraviolet light treatment to help relieve your itching.

Other ways to reduce itching include:

• Avoiding hot baths. Cooler water can limit irritation to your skin.
• Gently patting yourself dry after bathing. Vigorous rubbing with a towel can irritate your skin.
• Taking starch baths. Add half a box of starch to a tub of lukewarm water. This can help soothe your skin.

Experimental Treatments

Researchers are studying other treatments for PV. An experimental treatment for itching involves taking low doses of selective serotonin reuptake inhibitors (SSRIs). This type of medicine is used to treat depression. In clinical trials, SSRIs reduced itching in people who had PV.

Imatinib mesylate is a medicine that’s approved for treating leukemia. In clinical trials, this medicine helped reduce the need for phlebotomy in people who had PV. This medicine also helped reduce the size of enlarged spleens.

Researchers also are trying to find a treatment that can block or limit the effects of an abnormal JAK2 gene. (A mutation, or change, in the JAK2 gene is the major cause of PV.)

Source: National Heart, Lung, and Blood Institute, National Institutes of Health.

Polycythemia Vera: What’s the Treatment?

Written by Stephanie Watson

• Phlebotomy
• Medicines
• Radiation Therapy
• To Feel Better During Treatment

Polycythemia vera (PV) causes your bone marrow to make too many red blood cells. You need these cells to carry oxygen around your body, but too many of them can make your blood thicken and form clots. Sometimes blood clots lead to a heart attack or stroke.

Treatments lower the number of red blood cells and prevent blood clots. They also relieve other symptoms, like headaches and vision problems. With the right treatment, you can stay healthy — and feel better.

This is the main PV treatment. It removes some of your blood to get rid of extra red blood cells. It also lowers your red blood cell count, so your blood thickness starts to get closer to normal.

You may get phlebotomy when your doctor first tells you that you have PV. It’s a lot like donating blood. The doctor or nurse will put a needle into a vein in your arm and remove a small amount of blood from your body.

The goal is to lower your hematocrit level. Hematocrit is the percentage of red blood cells compared with the total amount of blood.

You’ll get this treatment once a week or month until your hematocrit goes down to around 45%. Once your red blood cell level has dropped, you’ll get phlebotomy less often.

After your red blood cell level drops, you’ll take one of these prescription drugs to slow the production of new red blood cells in your bone marrow.

Hydroxyurea is a cancer drug that slows down the growth of new cells in your body. In PV, it lowers the number of red blood cells and platelets, which are cells that help blood clot. If you take this drug, you may not need phlebotomy. Hydroxyurea can also prevent or treat an enlarged spleen — a complication of PV.

Hydroxyurea comes in a capsule that you take by mouth. It can cause side effects, such as:

• Nausea
• Vomiting
• Constipation
• Diarrhea
• Rash

It’s very unlikely, but it may cause cells to turn cancerous. Though the risk is very low, your doctor should check you for cancer while you take hydroxyurea.

Interferon-alfa (Intron A) works on your immune system. It targets quickly dividing red blood cells to slow their production.

You take interferon-alfa as a shot just under your skin. Some possible side effects from interferon-alfa are:

• Flu-like symptoms — fever, chills, and muscle aches
• Tiredness
• Nausea, vomiting

A long-acting version, called peginterferon alfa (Pegasys), has fewer side effects.

Ropeginterferon alfa-2b-njft (Besremi) is the first interferon therapy specifically approved for polycythemia vera. It attaches to interferon alfa/beta receptors (IFNAR), triggering several reactions that cause the bone marrow to produce fewer red blood cells. Administered by injection, it is administered every two weeks.

Some possible side effects include:

• Joint pain
• Flu-like symptoms 
• Fatigue
• Itching
• Urinary tract infection
• Depression
• Transient ischemic attacks (stroke-like attacks). 

Aspirin thins your blood and prevents clots. It also eases symptoms such as burning in your hands and feet, itching, and bone pain. Your doctor might recommend that you take a low dose of aspirin every day.

Daily aspirin use has some risks. It can make bleeding more likely, especially in the stomach and other parts of your digestive system. Talk to your doctor about these and other risks before you start to take aspirin regularly.

Your doctor may also consider other treatments such as anagrelide, busulfan (Myleran), imatinib (Gleevec), and ruxolitinib (Jakafi). These medicines help certain types of blood cells work better, so your blood flow improves and is less thick. They might be an option if other treatments don’t work or if you can’t take drugs like hydroxyurea and interferon-alfa. Each drug is different, so your doctor will talk with you about side effects and what the latest research shows.

This treatment slows red blood cell production in bone marrow, which thins your blood and helps it flow more easily. Yet doctors don’t use radiation therapy very often for PV because it also can make blood cancer (leukemia) more likely.

In addition to your medical plan, use these self-care tips to help you feel better:

1. Exercise daily. Staying active will help keep your blood flowing and prevent clots. Also stretch regularly — especially your legs and ankles — to improve blood flow.
2. Take cool baths to prevent skin irritation. Gently pat yourself dry afterward — don’t rub your skin.
3. Keep warm. PV affects your blood flow, which can cause you to lose heat in your hands and feet. Wear gloves and socks when it’s cold outside.
4. Drink a lot of water. Extra fluids will help prevent your blood from getting too thick.
5. Try not to hurt your feet. Wear shoes to protect against skin damage. Check your feet often for any cuts or sores.
6. Treat itchy skin. Moisturize or use an over-the-counter antihistamine.
7. Shield your skin from the sun. Apply an SPF 30 or higher sunscreen every time you go outside. Also wear sun-protective clothing, including a wide-brimmed hat.

It’s normal to have a mix of emotions when you have a serious condition. If your concerns start to worry you or get you down, talk to your doctor, a therapist or counselor, or a support group. Let close friends or family members know what you’re going through and how they can help.

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Bloodletting (hemoexfusion) 1 procedure

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Hemoexfusion is a procedure for activating the body’s defenses by removing a volume of blood safe for human health from it through a vein puncture. This helps to reduce blood viscosity, renew its composition and improve metabolism.

Indications for examination

• Hemosiderosis – excessive deposition in the tissues of hemosiderin, which is a dark yellow pigment, which consists of iron oxide. Symptoms of hemosiderosis: appearance on the skin of pigmented spots or hemorrhagic rash of brick red color;
• Hemochromatosis is a pathology in which a violation of iron metabolism provokes its accumulation in the body. Symptoms of hemochromatosis: chronic fatigue, increased skin pigmentation, joint pain, hypothyroidism;
• Polycythemia vera is a benign tumor process of the blood system that provokes an increase in the mass of circulating blood. Symptoms of polycythemia: general weakness, high blood pressure, heaviness in the abdomen, enlargement of the spleen and liver;
• Secondary erythrocytosis is an increase in the content of red blood cells in the blood, as a result of which the blood becomes more viscous. Symptoms of secondary erythrocytosis: mental decline, dizziness, headache, fatigue, visual disturbances

How is hemoexfusion treated?

Bloodletting treatment takes about 20-30 minutes. In this case, up to 450 ml of blood is taken. The volume of blood to be removed from the body is determined by our doctor, taking into account the patient’s age, general condition and specific diagnosis.

Benefits of bloodletting

Hemoexfusion , carried out under the supervision of specialists from our medical center, will help prevent the development of many complications associated with damage to internal organs. The result of this procedure will be a decrease in high blood pressure, a renewal of the blood composition and an improvement in overall well-being.

Examination preparation

For the procedure, you must have a referral from the attending physician certified by his signature and seal on the letterhead of the medical institution.
The direction should indicate: the volume of blood to be removed, the need for replacement with saline or another drug (if necessary, its amount in ml). Referral is valid for 6 months.
Special preparation before using this method is not required. If there is no referral from the attending physician, then you can consult with our specialist to identify indications and contraindications for this medical procedure.
To do this, you may need to take laboratory tests (blood, urine) and undergo an ultrasound of the internal organs.

• Anemia;
• Hypotension;
• Cirrhosis of the liver;
• Hypoproteinemia;
• Oncological diseases;
• Blood clotting disorders;
• Phlebitis of peripheral veins;
• Severe cardiovascular diseases.

It is not recommended to use this method of treatment for women during menstrual bleeding or in early pregnancy, people with mental illness and people who are intoxicated.

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Consult a specialist for possible contraindications

High blood pressure or hypertension

The article was prepared by the therapist of the Center for Immunology and Reproduction Boyko Tatiana Valentinovna

One of the main consequences of a prolonged increase in pressure in the arteries is damage to the internal organs (target organs).

Target organs:

Heart. Manifestations: angina pectoris (a form of coronary heart disease, manifested by retrosternal pain during exercise), myocardial infarction, heart failure, sudden cardiac death.

Brain. Manifestations: thrombosis, hemorrhages, hypertensive encephalopathy (damage to cerebral vessels with prolonged increase in blood pressure), cerebral lacunae (small hemorrhages in the brain).

Kidneys. Manifestations: microalbuminuria, proteinuria (the appearance of protein in the urine), chronic renal failure (significant impairment of kidney function).

Vessels. Retinal vessels (retinopathy – damage to the vessels of the retina), carotid arteries, aortic aneurysm (saccular dilatation of the aorta).

Metabolic syndrome is a group of conditions accompanied by metabolic disorders, accompanied by obesity, elevated triglycerides, low levels of high density lipoproteins, high blood pressure, high insulin levels, insulin resistance.
The more components of the metabolic syndrome are present, the more likely it is to develop complications – the development of diabetes, heart and brain damage.

In our clinic, you can be examined for polymorphism of vascular tone genes, which is necessary not only to determine the cause of arterial hypertension, but also to properly treat this disease. In addition to arterial hypertension, there are other reasons that serve as a reason for conducting a genetic study. Simultaneously with this profile, an assessment of the risk of developing thrombophilic complications is carried out – the most important component of the development of heart attacks, strokes, and thromboembolism. The profile is called Polymorphism of the genes of the hemostasis system. Genetic tests are given once in a lifetime and allow you to assess the risk of developing a particular pathology. In this case (polymorphism of genes of the hemostasis system and polymorphism of genes of vascular tone), vascular disorders and thrombosis. You can read more about each of them on the pages of these analyses.

Additional studies are needed to make a correct diagnosis and develop treatment tactics.

In the presence of disorders of sugar metabolism or a high risk of disorders (elevated glucose levels, symptoms of diabetes mellitus, diabetes in relatives)

Additional tests your doctor may order for hypertension:
Blood test for renin and angiotensin I
Aldosterone blood test

How to measure blood pressure correctly, why measure pressure on both hands

Blood pressure should be measured 1-2 hours after a meal. Within an hour before the measurement, refrain from smoking, eating, coffee, strong tea. Blood pressure should be measured in a sitting position, after a 5-minute rest, without straining the muscles of the arms and legs, without holding your breath during the procedure and without talking to others; The arm on which blood pressure is measured should be at the level of the heart. Measurement of blood pressure should be taken on both arms and focus on the higher measurement.

Which blood pressure numbers are normal and which can be considered high blood pressure

The numbers of normal blood pressure (BP) range from 90/60 to 139/89 mm Hg, less than 90/60 – this is arterial hypotension , i.e. decrease in blood pressure, and above 139/89 – this is arterial hypertension – increased blood pressure.

The first number means systolic blood pressure, which depends on the strength and speed of the contraction of the heart, it is also called “upper”. The second figure – diastolic blood pressure, depends on the tone and elasticity of the vessels, another name is “lower”.

How to deal with high blood pressure, how to reduce blood pressure

A sharp increase in blood pressure is called a hypertensive crisis – this is not only a sharp increase in blood pressure to high numbers, but also an increase in all the main symptoms of the disease.

The reasons for the development of hypertensive crises are mental trauma and unrest, negative emotions, weather changes (changes in atmospheric pressure, temperature and air humidity).

Signs of a hypertensive crisis

Often crises are accompanied by a sharp headache, dizziness, nausea, vomiting, loss of consciousness, visual disturbances (fog before the eyes, flashing “flies”). Most often, hypertensive crises are observed when, after feeling better, the patient stops regularly taking antihypertensive drugs (drugs that reduce pressure).

First of all, you need to calm down and lie down. Open a window and provide fresh air. It is necessary to observe strict bed rest. Sometimes it is easier in a sitting position.

If necessary, call an ambulance.

Before the arrival of the medical team, the so-called distraction procedures should be carried out: put a mustard plaster on the occipital region and calf muscles or make hot foot baths to drain blood to the legs. If a hypertensive crisis is accompanied by pain in the region of the heart, then you can take validol or nitroglycerin under the tongue.

It is recommended to take additional doses of the medicine that the patient usually took. You can put tablets under the tongue to lower blood pressure. As prescribed by the doctor, the patient may be given injections of pressure-lowering drugs.

If during a hypertensive crisis the patient is in an excited state, i.e., a neurovegetative crisis occurs , then he is shown the introduction of sedatives that relieve the excited state.

In any case, it is necessary to reduce blood pressure gradually, not to normal, but to a more comfortable state.

After the removal of the hypertensive crisis, they proceed to planned treatment with medications. If a hypertensive crisis has developed against the background of regular antihypertensive therapy, then it is necessary to reconsider the drugs of choice for the treatment of a particular patient. Only a doctor can do this. Therefore, after removing the crisis, you should seek help from your doctor.

Why is it necessary to measure the pressure during pregnancy, as evidenced by high blood pressure

Hypertension is the most common complication of pregnancy and causes maternal and infant morbidity and mortality worldwide.

High blood pressure in early pregnancy is the most dangerous symptom. In this condition, the walls of the blood vessels are very narrowed, and this interferes with the normal supply of oxygen and other nutrients from the blood to the developing fetus, which means that the physiological growth of the embryo slows down. Excessively high blood pressure can also cause spontaneous miscarriage.

Also, hypertension can provoke a delay in the development of the fetus, placental insufficiency, bleeding, chronic hypoxia (lack of oxygen), premature detachment of the placenta.

High blood pressure during pregnancy may be a symptom of complications such as preeclampsia is a condition that occurs in the second half of pregnancy and is manifested by a triad of symptoms: high blood pressure, edema and the appearance of protein in the urine.

Blood pressure products

1. alcoholic and carbonated drinks;
2. spicy, smoked, salty foods and prepared meals, preserves;
3. foods rich in caffeine: cocoa, coffee, strong tea;
4. fish oil, fatty fish and meats, ice cream;
5. cakes and pastries containing buttercream, buns;
6. products with a high content of starchy substances: semolina, potatoes, white flour products;
7. practically all by-products: liver, brains, kidneys;
8. seasonings and spices: pepper (black and red), raw onion, horseradish, mustard, cloves.

Salt. Table salt, as you know, triggers mechanisms in the body that lead to spasm of blood vessels. In addition, it is salt that makes a person feel thirsty, “forcing” him to consume more liquid, which leads to an increase in blood volume and an increase in blood pressure.

Pressure reducing products

1. Products containing calcium are an active ingredient for reducing pressure, and dairy products are the best suppliers of it, as they contain vitamin D, which improves the absorption of calcium, cheese and yogurt, including; and also, green vegetables, broccoli, tofu and almonds.