Polycythemia Vera :Causes-Symptoms-Diagnosis-Treatment

What is Polycythemia Vera(PV)?

Polycythemia vera is an unprecedented blood ailment wherein there is a boom in all blood cells, particularly crimson blood cells. The growth in blood cells makes your blood thicker. This can cause strokes or tissue and organ damage.

Polycythemia vera (PV) is an unprecedented type of blood in most cancers wherein your body produces too many pink blood cells.

Red blood cells carry oxygen for the duration of your body. When you have got too many pink blood cells, your blood thickens and flows more slowly. The pink blood cells can clump together and shape clots inside your blood vessels.

If it's not dealt with, PV can lead to existence threatening headaches. Blood that flows more slowly can lessen the quantity of oxygen that reaches your heart, brain, and different important organs. And blood clots can absolutely block blood float inside a blood vessel, inflicting a stroke or even demise.

What is Polycythemia Vera(PV)?
Polycythemia Vera

In the long term, PV can lead to scarring of the bone marrow, referred to as myelofibrosis, as well as leukemia, every other sort of blood most cancers.

There’s no treatment for PV, but you can manage the situation with remedy. Your physician will probably do ordinary blood draws and prescribe medication to assist prevent extreme blood clots. It’s crucial to speak together with your doctor if you’re vulnerable to PV and have any of its signs.

  1. Heart

  2. Arteries

  3. Veins

  4. Blood vessels

Medical terms

  • Polycythemia vera (pol-e-sy-THEE-me-uh VEER-uh) is a type of blood for most cancers. It causes your bone marrow to make too many red blood cells. These excess cells thicken your blood, slowing its glide, which may also motive extreme troubles, consisting of blood clots.
  • Polycythemia vera is uncommon. It usually develops slowly, and you may have it for years without understanding. Often the circumstance is found for the duration of a blood check executed for another cause.
  • Without remedy, polycythemia vera can be existence-threatening. But right hospital treatment can help ease signs, signs and headaches of this disorder.Polycythemia vera is an extraordinary, persistent sickness related to the overproduction of blood cells within the bone marrow (myeloproliferative). The overproduction of pink blood cells is maximum dramatic, however the production of white blood cells and platelets are also expanded in most cases. Since red blood cells are overproduced within the marrow, this leads to abnormally high numbers of circulating red blood cells (red blood mass) in the blood. Consequently, the blood thickens and will increase in quantity, a circumstance called hyperviscosity. Thickened blood might not float via smaller blood vessels well. A sort of signs can arise in people with polycythemia vera inclusive of nonspecific signs together with headaches, fatigue, weak spot, dizziness or itchy skin; an enlarged spleen (splenomegaly); an expansion of gastrointestinal problems; and the danger of blood clot formation, which may prevent blood go with the flow to crucial organs. More than ninety percent of individuals with polycythemia vera have a variant (mutation) inside the JAK2 gene. The actual function that this modification performs within the development of polycythemia vera isn't but acknowledged.

Polycythemia vera changed into first suggested inside the clinical literature in 1892. The time period “myeloproliferative ailment” (MPD) became first used to define polycythemia vera and associated issues in 1951. In 2008, the World Health Organization reclassified MPDs to “myeloproliferative neoplasms” (MPNs) to reflect the consensus that those illnesses are blood cancers (neoplasms). This institution of disorders is characterized by the overproduction (proliferation) of 1 or extra of the three important blood cell lines – pink or white blood cells or platelets. Red blood cells convey oxygen to the frame. White blood cells fight contamination. Platelets are involved in clotting of the blood in response to harm. Three different problems are usually labeled as MPNs: chronic myeloid leukemia, essential thrombocythemia and idiopathic myelofibrosis. Because MPNs are characterized by means of uncontrolled cellular boom, they'll also be categorized as blood cancers.

Symptoms Polycythemia vera (PV)

The signs and symptoms of polycythemia vera generally broaden slowly over a few years. Often, the ailment is discovered incidentally on a blood take a look at as part of a habitual exam earlier than considerable signs occur. Occasionally, affected individuals may file vague, nonspecific symptoms that finally cause analysis of the disease.

Many individuals with polycythemia vera slowly development a selection of trendy, nonspecific symptoms that are not unusual to many disorders which includes headaches, fatigue, weak spot, dizziness, immoderate sweating especially at night, and itchy pores and skin that, in extreme instances, can be worse after getting showered or a heat bath. Additional signs may also arise in a few affected individuals including blurred vision, ringing within the ears (tinnitus), and strange redness of the skin mainly on the face.

Eventually, the spleen becomes involved. The spleen is an organ located in the top left part of the abdomen that filters out worn out blood cells. It regularly becomes abnormally enlarged in individuals with polycythemia vera as it tries to clear a greater number of blood cells than regular – a circumstance referred to as splenomegaly. Splenomegaly may motivate an affected individual to have a bloated or complete feeling in the stomach.

Less not unusual symptoms related to polycythemia vera encompass an inclination to bruise without difficulty, frequent nosebleeds or bleeding from the gums, growth of the liver (hepatomegaly), and erythromelalgia, a condition characterized through a reddened or purplish appearance to the pores and skin of the fingers and ft. The skin may feel warm to the touch. Erythromelalgia also can cause a painful, burning sensation or swelling of the affected areas.

Some individuals with polycythemia vera may additionally increase signs secondary to reduced blood flow (because of thickening of the blood) and abnormalities affecting the platelets, that could increase someone’s risk of developing blood clots. Complications that arise due to blood clots may be referred to as thrombotic occasions and, in rare cases, can be the primary obvious signal of polycythemia vera. Specific signs and symptoms depend upon where a blood clot forms. A blood clot can cause a stroke, chest pain (angina), a coronary heart assault, deep vein thrombosis (DVT) or a pulmonary embolism. DVT occurs when a blood clot forms in the legs may additionally cause the legs to end up painful and swollen. A pulmonary embolism happens when a clot bureaucracy inside the lungs or while a bit of a DVT breaks off and travels through the bloodstream ultimately becoming stuck within the pulmonary artery. A pulmonary embolism can cause breathlessness, an unexpected pain in the chest, exhaustion, or lifestyle-threatening complications which includes excessive blood stress of the pulmonary artery.

Some individuals with polycythemia vera have advanced Budd-Chiari syndrome, a situation wherein a blood clot paperwork inside the most important blood vessel leading to the liver (hepatic vein thrombosis). Symptoms of Budd-Chiari syndrome consist of pain in the upper right part of the stomach, an abnormally enlarged liver (hepatomegaly), yellowing of the pores and skin and the whites of the eyes (jaundice), and/or accumulation of fluid within the area (peritoneal cavity) among the two layers of the membrane that line the stomach (ascites).

The ordinary proliferation of red blood cells might also motivate peptic ulcers, gout, and kidney stones. Peptic ulcers are open sores on the lining of the gastrointestinal tract, which might also reason bleeding (hemorrhaging) inside the gastrointestinal tract. Gout is an inflammation of the joints due to a build-up of uric acid. Abnormally excessive degrees of uric acid also can cause kidney stones. Gout and kidney stones related to polycythemia vera arise because of the high turnover of purple blood cells, which leads to higher-than-regular uric acid manufacturing.

Polycythemia vera may subsequently “burn out” so that scar tissue replaces the marrow and the ailment resembles idiopathic myelofibrosis. This can also be known as the “spent phase” of polycythemia vera. When this occurs, the marrow can not produce blood cells resulting in low stages of healthy, functioning purple blood cells (anemia), platelets (thrombocytopenia) and white blood cells (leukopenia). In uncommon cases, polycythemia vera may also sooner or later develop into a form of leukemia known as acute myeloid leukemia.

Many people with polycythemia vera do not have significant signs and symptoms or signs and symptoms. Some human beings may have vague signs and symptoms including headache, dizziness, fatigue and blurred vision.

More-specific symptoms of polycythemia vera include:

  • Itchiness, especially after a warm bath or shower

  • Numbness, tingling, burning, or weakness in your hands, feet, arms or legs

  • A feeling of fullness soon after eating and bloating or pain in your left upper abdomen due to an enlarged spleen

  • Unusual bleeding, such as a nosebleed or bleeding gums

  • Painful swelling of one joint, often the big toe

  • Shortness of breath and difficulty breathing when lying down

When to see a doctor

Make an appointment with your physician if you have signs or symptoms of polycythemia vera.

Causes Polycythemia vera (PV)

Polycythemia vera takes place when a mutation in a gene causes a problem with blood cellular manufacturing. Normally, your frame regulates the wide variety of each of the 3 varieties of blood cells you have got — red blood cells, white blood cells and platelets. But in polycythemia vera, your bone marrow makes too many of those blood cells.

The motive of the gene mutation in polycythemia vera is unknown, however it's usually not inherited from your dad and mom.Polycythemia vera takes place greater frequently in guys than in women. You’re more likely to get PV after age 60, however it is able to start at any age.

Mutations (modifications) to the JAK2 gene are the principal reason for the ailment. This gene controls manufacturing of a protein that helps make blood cells. About ninety five percent of humans with PV have this kind of mutation.

The mutation that causes PV may be passed down through households. But more regularly, it can take place without any family connection. Research is ongoing into the purpose of the genetic mutation in the back of PV.

If you have PV, your chance for growing critical headaches relies upon on how probable you are to broaden a blood clot. Factors that can boom your hazard of developing blood clots in PV consist of:

  • a history of blood clots

  • being over age 60

  • high blood pressure

  • diabetes

  • smoking

  • high cholesterol

  • pregnancy

Blood that’s thicker than normal can always increase your risk of blood clots, no matter the cause.

Risk factors Polycythemia vera (PV)

Polycythemia vera can arise at any age, but it's more commonplace in adults among 50 and 75. Men are more likely to get polycythemia vera, however women have a tendency to get the disorder at more youthful ages.

What is the life expectancy of someone with polycythemia vera?

The life expectancy of someone with polycythemia vera depends on the type and severity of the disease If a person has mild polycythemia vera he can live normal life span However if it is severe he might have to undergo treatment to reduce the number of red blood cells so that his health is not at risk.

What foods to avoid if you have polycythemia?

Polycythemia is a condition in which the body makes too many red blood cells As a result the blood becomes thicker and can thicken other bodily fluids such as the fluid that cushions your joints This may cause pain at rest and/or when you move Additional symptoms of polycythemia include easy bruising nosebleeds headaches and shortness of breath To help combat this disease avoid eating spicy foods such as onions and garlic because they promote bleeding Also avoid drinking alcohol or caffeine because they can cause your blood to become thinner than normal and promote bleeding If you are taking any medications or supplements that thin your blood it is important.

polycythemia vera is not cancer

Polycythemia vera is a type of blood cancer that causes the bone marrow to make too many red blood cells Many people with polycythemia vera have no symptoms but those who do have symptoms may experience fatigue shortness of breath and leg swelling Treatment for polycythemia vera varies based on how advanced the disease is and whether it has spread to other parts of the body.

Complications Polycythemia Vera

Possible complications of polycythemia vera include:

  • Blood clots. Increased blood thickness and decreased blood waft, in addition to abnormalities to your platelets, enhance your danger of blood clots. Blood clots can cause a stroke, a heart attack, or a blockage in an artery in your lungs or a vein deep within a leg muscle or within the abdomen.

  • Enlarged spleen. Your spleen facilitates your body to fight contamination and filter unwanted fabric, together with old or damaged blood cells. The multiplied variety of blood cells due to polycythemia vera makes your spleen paintings tougher than ordinary, which causes it to amplify.

  • Problems due to high levels of red blood cells. Too many pink blood cells can result in a number of other complications, along with open sores at the interior lining of your belly, higher small intestine or esophagus (peptic ulcers) and irritation for your joints (gout).

  • Other blood disorders. In rare instances, polycythemia vera can cause different blood diseases, which include a modern disorder wherein bone marrow is changed with scar tissue, a situation wherein stem cells don't mature or characteristic nicely, or cancer of the blood and bone marrow (acute leukemia).

Diagnosis Polycythemia vera (PV)

Diagnosis of polycythemia vera may be made based upon a radical scientific evaluation, exact patient history, and various specialized assessments. In many instances, the sickness can be detected from blood assessments conducted at some stage in a routine exam. An entire blood memory (CBC) may additionally exhibit expanded numbers of pink blood cells and every so often platelets and white blood cells.

Blood tests may additionally degree hemoglobin and hematocrit. Hemoglobin is the protein inside pink blood cells that carry oxygen. Hematocrit is the share of pink blood cells inside the overall blood quantity. If those measures are improved it is able to imply polycythemia vera.

Physicians may additionally measure the ranges of erythropoietin (EPO), a hormone that causes the bone marrow to supply purple blood cells. In individuals with polycythemia vera, EPO stages are abnormally low. This take a look at is typically achieved to differentiate polycythemia vera from secondary polycythemia, wherein EPO ranges aren't affected.

In a few cases, surgical removal and microscopic exam of bone marrow tissue (biopsy) may also be used to diagnose polycythemia vera. The sample tissue is tested to determine whether or not the marrow is functioning well.

A kind of specialized check may be used to pick out the JAK2 mutation in blood cells, which is also diagnostic of polycythemia vera.

Your doctor will take a detailed medical history and perform a physical exam.

Blood tests

If you have polycythemia vera, blood tests might reveal:

  • More red blood cells than normal and, sometimes, an increase in platelets or white blood cells

  • A greater percentage of red blood cells that make up total blood volume (hematocrit measurement)

  • Elevated levels of the iron-rich protein in red blood cells that carries oxygen (hemoglobin)

Bone marrow aspiration or biopsy

If your physician suspects that you have polycythemia vera, she or he might advise collecting a pattern of your bone marrow through a bone marrow aspiration or biopsy.

A bone marrow biopsy entails taking a pattern of stable bone marrow cloth. A bone marrow aspiration is generally executed at the same time. During an aspiration, your health practitioner withdraws a pattern of the liquid portion of your marrow.

Specific gene testing

If you have got polycythemia vera, evaluation of your bone marrow or blood might display the gene mutation associated with the disorder.

Treatment Polycythemia vera (PV)

The treatment of polycythemia vera is aimed at lowering the tiers of purple blood cells and stopping the complications of the disorder, mainly blood clot (thrombosis) formation. Treatment options encompass phlebotomy and drug therapy.

Most individuals with polycythemia vera can have their blood drawn (much like as is carried out when donating blood) usually at ordinary intervals over several months. Phlebotomy is used to lessen the extent of circulating crimson blood cells so that blood can flow and function well. Phlebotomy may additionally solve signs associated with thickened blood and extended red blood cell production. Phlebotomy can be the handiest remedy necessary for a few human beings, for decades. However, this method does not treat elevated platelet stages (thrombocythemia), extended white blood cellular ranges (leukocytosis), itchy pores and skin or gout. In some instances, phlebotomy may contribute to accelerated platelet ranges. Phlebotomy is also known as venesection.

Many individuals with polycythemia vera will also acquire treatment with certain capsules that suppress the formation of blood cells via the marrow (myelosuppressive drugs). A chemotherapy drug called hydroxyurea is most often used. Another chemotherapy drug used is busulfan. Other pills along with chlorambucil and radioactive phosphorus had been used inside the beyond, however these tablets, mainly in individuals requiring long-term remedy, were related to improved chance of developing leukemia.

Anagrelide is a drug used to decrease the wide variety of platelets and reduce the chance of blood clot formation. Another drug, referred to as interferon alfa, stimulates the immune device to suppress blood mobile manufacturing.

Additional cures used for polycythemia vera encompass low-dose aspirin to lower the risk of blood clot formation, a drug called allopurinol to treat high uric acid ranges, and antihistamines or ultraviolet light therapy to deal with excessive, persistent itchiness.

Individuals who enter the “spent segment” of polycythemia vera, in which the bone marrow does not produce wholesome, functioning blood cells, might also require periodic blood transfusions to preserve enough degrees of blood cells. During the spent phase, the spleen may additionally emerge as extensively enlarged and painful, potentially requiring its removal through surgical operation (splenectomy).

Jakafi (ruxolitinib) became authorized by using the FDA in 2011 for treatment of sufferers with intermediate or excessive danger of myelofibrosis, such as post-polycythemia vera myelofibrosis. In 2014, Jakafi became approved for PV sufferers who've had an inadequate reaction to or are illiberal of hydroxyurea. This medication inhibits JAK 1 and a couple of enzymes which can be used in regulating blood and immunological functioning. Jakafi is manufactured through Incyte Corp.

There's no remedy for polycythemia vera. Treatment specializes in lowering your chance of headaches. These treatments may additionally ease your symptoms.

Blood withdrawals

The maximum common treatment for polycythemia vera is having frequent blood withdrawals, using a needle in a vein (phlebotomy). It's the same system used for donating blood.

This decreases your blood extent and decreases the number of extra blood cells. How frequently you need to have blood drawn depends on the severity of your condition.

Treatments to reduce itching

If you have got bothersome itching, your health practitioner might also prescribe medicine, inclusive of antihistamines, or advocate ultraviolet light treatment to alleviate your pain.

Medications which can be normally used to treat depression, called selective serotonin reuptake inhibitors (SSRIs), helped relieve itching in scientific trials. Examples of SSRIs include paroxetine (Brisdelle, Paxil, Pexeva, others) or fluoxetine (Prozac, Sarafem, Selfemra, others).

Drugs that reduce the number of red blood cells

If phlebotomy by myself does not help sufficiently, your health practitioner may additionally propose medicinal drugs that may reduce the number of purple blood cells to your bloodstream. Examples include:

  • Hydroxyurea (Droxia, Hydrea)

  • Interferon alfa-2b (Intron A)

  • Ruxolitinib (Jakafi)

  • Busulfan (Busulfex, Myleran)

Heart medications

Your physician may also probably prescribe medicines to control hazard factors for coronary heart and blood vessel sickness, along with high blood pressure, diabetes and atypical ldl cholesterol.

Your physician may also propose that you take a low dose of aspirin to lessen your risk of blood clots. Low-dose aspirin may also assist reduce burning pain in your feet or arms.

Lifestyle and home remedies

You can take steps to assist yourself feel better in case you've been recognized with polycythemia vera. Try to:

  • Exercise. Moderate exercising, consisting of strolling, can improve your blood glide. This enables decrease your hazard of blood clots. Leg and ankle stretches and physical activities also can improve your blood move.

  • Avoid tobacco. Using tobacco can purpose your blood vessels to slim, increasing the chance of heart assault or stroke due to blood clots.

  • Avoid low-oxygen environments. Living at high altitudes, skiing or mountaineering in mountains all lessen the oxygen degrees on your blood even in addition.

  • Be good to your skin. To lessen itching, shower in cool water, use a mild cleaner and pat your pores and skin dry. Adding starch, which includes cornstarch, for your tub would possibly help. Avoid hot tubs, heated whirlpools, and warm showers or baths.
    Try not to scratch, as it can harm your skin and increase the threat of infection. Use lotion to hold your pores and skin moist.

  • Avoid extreme temperatures. Poor blood glide will increase your threat of injury from warm and bloodless temperatures. In bloodless weather, usually wear warm clothing, especially on your fingers and feet. In hot weather, protect yourself from the sun and drink masses of liquids.

  • Watch for sores. Poor move could make it hard for sores to heal, specifically on your fingers and feet. Inspect your ft frequently and inform your physician approximately any sores.

Preparing for your appointment

You're possibly starting by seeing your primary care medical doctor. If you're identified with polycythemia vera, you are probably noted as a health practitioner who specializes in blood conditions (hematologist).

Here's a few information to help you get prepared for your appointment.

What you can do

Make a list of:

  • Your symptoms, Which include any that seem unrelated to the cause for which you scheduled the appointment, and when they started

  • Key personal information, including other medical conditions and family medical history

  • All medications, vitamins or supplements you take, including doses

  • Questions to ask your doctor

For polycythemia vera, questions to ask your doctor include:

  • What's the most likely cause of my symptoms?

  • What tests do I need?

  • Is this condition temporary, or will I always have it?

  • What treatments are available, and which do you recommend?

  • I have other health conditions. How can I best manage them together?

  • Should I see a specialist?

  • Will I need follow-up visits? If so, how often?

  • Are there brochures or other printed material I can have? What websites do you recommend?

Don't hesitate to invite different questions you think of at some stage in the appointment. Take a member of the family or friend alongside, if viable, that will help you not forget the records you're given.

What to expect from your doctor

Your doctor is likely to ask you questions, including:

  • Have your symptoms been continuous or occasional?

  • How severe are your symptoms?

  • What, if anything, seems to improve your symptoms?

  • Does anything worsen your symptoms?

General summary

  1. Polycythemia Vera known as PV is a rare disorder that causes an abnormal increase in the production of red blood cells These extra red blood cells can clog small blood vessels throughout the body limiting the delivery of needed oxygen to organs and tissues Without treatment people with PV can develop severe life-threatening complications.
  2. Folic acid is a water-soluble B vitamin that helps the body turn food into energy Folate also plays an important role in red blood cell formation because it is necessary to produce new cells.

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