Do You Have Polycythemia Vera?

While it’s never pleasant to think you may have a serious disease it’s important that you find out early on. Some diseases are so subtle and asymptomatic you need tests to discover them. Polycythemia vera is considered a rare disease with about 2 in every 100,000 people having the disease. It usually develops slowly, and you might have it for years without knowing. Often the condition is found during a blood test done for another reason. Do you have Polycythemia Vera?

Polycythemia Vera

 

WARNING: THIS POST CONTAINS A GRAPHIC VIDEO OF A BONE MARROW BIOPSY/ASPIRATION

Polycythemia vera (pol-e-sy-THEE-me-uh VEER-uh) is a chronic, progressive myeloproliferative neoplasm (MPN). PV is a slow-growing blood cancer in which your bone marrow makes too many red blood cells. These excess cells thicken your blood, slowing its flow. They also cause complications, such as blood clots, which can lead to a heart attack or stroke.

Without treatment, polycythemia vera can be life-threatening. But proper medical care can help ease signs, symptoms and complications of this disease. Over time, in some cases there’s a risk of progressing to more-serious blood cancers, such as myelofibrosis or acute leukemia.

Myelofibrosis is a serious bone marrow disorder that disrupts your body’s normal production of blood cells. The result is extensive scarring in your bone marrow, leading to severe anemia, weakness, fatigue and often an enlarged spleen. It is an uncommon type of chronic leukemia which is a cancer that affects the blood-forming tissues in the body. Myelofibrosis one of the myeloproliferative disorders. Many people with myelofibrosis get progressively worse. Some may eventually develop a more serious form of leukemia. Yet it’s also possible to have myelofibrosis and live symptom-free for years.

Polycythemia Vera

Polycythemia vera Symptoms:

Like patients with other types of MPNs, PV patients may present with a wide range of symptoms or exhibit no symptoms at all. There are some common symptoms of the disease –

  • Headache
  • Sweating
  • Ringing in the ears
  • Blurred vision or blind spots
  • Dizziness or vertigo
  • Reddish or purplish skin
  • Unexpected weight loss
  • Bleeding or clotting
  • Early feeling of fullness (satiety)
  • Itching (pruritis), especially after taking a shower
  • Burning and redness of the hands or feet
  • Tiredness (fatigue)
  • Night sweats
  • Bone pain

 

Tests for Polycythemia Vera:

Blood Tests:
A complete blood count can identify an increase in hemoglobin as well as white blood cells and/or platelets.

Bone Marrow Biopsy& Aspiration:
These are procedures to collect and examine bone marrow which is the spongy tissue inside some of your larger bones. A bone marrow biopsy & aspiration may reveal an excess of precursors to red blood platelets, red blood cells and white blood cells.

Gene Mutation Analysis of Blood Cells:
Approximately 95% of PV patients test positive for a JAK2 gene mutation. An analysis of red blood cells may also indicate a low erythropoietin level (a hormone involved in red blood cell production).

Low Erythropoietin Level:
An analysis of red blood cells may also indicate a low erythropoietin level (a hormone involved in red blood cell production).

 

Treatments:

  • Taking blood out of your veins. Drawing some blood out of your veins in a procedure called phlebotomy is usually the first treatment option for people with polycythemia vera. This reduces the number of blood cells and decreases your blood volume, making it easier for your blood to function. How often you need phlebotomy depends on the severity of your condition.
  • Low-dose aspirin. Your doctor may recommend that you take a low dose of aspirin to reduce your risk of blood clots. Low-dose aspirin may also help reduce burning pain in your feet or hands.
  • Medication to decrease blood cells. For people with polycythemia vera who aren’t helped by phlebotomy alone, medications, such as hydroxyurea (Droxia, Hydrea) which are chemotherapy drug. These drugs are used to suppress your bone marrow’s ability to produce blood cells might be used.
  • Medication to destroy cancer cells. Ruxolitinib (Jakafi) is approved by the Food and Drug Administration to treat people with polycythemia vera who don’t respond to or can’t take hydroxyurea. It helps your immune system destroy cancer cells, and can improve some polycythemia vera symptoms.
  • Therapy to reduce itching. If you have bothersome itching, your doctor may prescribe medication, such as antihistamines, or recommend ultraviolet light treatment to relieve your discomfort.

Prognosis:

Approximately 15% of PV patients develop myelofibrosis, a progressive bone marrow disorder that results in bone marrow scarring, severe anemia, and enlargement of the liver and spleen. This change can be heralded by the onset of anemia, or a low red blood count, as opposed to high red blood counts, and a significant increase in the size of the spleen). In a smaller number of cases, PV may progress to acute leukemia (AML).

Additional complications that can occur with PV include arterial thromboses (heart attacks, strokes, intestinal gangrene), venous thromboses (of the portal and/or hepatic veins), or pulmonary embolism.

Patients at risk of developing these illnesses should have their blood counts routinely monitored and controlled by a physician.

Although Polycythemia vera is a very serious disease and some people need treatment beyond phlebotomy most patients enjoy a long life after diagnosis. But early detection is the key. Ask your doctor to test your blood if you have the symptoms of Polycythemia vera and even if you don’t! A simple blood test can tell answer the question; Do You Have Polycythemia Vera?

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