Polycythemia vera is a bone marrow disease that leads to an abnormal increase in the number of blood cells (primarily red blood cells).
Primary polycythemia; Polycythemia rubra vera; Myeloproliferative disorder; Erythremia; Splenomegalic polycythemia; Vaquez’s disease; Osler’s disease; Polycythemia with chronic cyanosis – Myelopathic polycythemia; Erythrocytosis megalosplenica; Cryptogenic polycythemia
Causes, incidence, and risk factors
Polycythemia vera is a disorder of the bone marrow. It mainly causes too much production of red blood cells, although the numbers of white blood cells and
It is a rare disease that occurs more often in men than women, and is rare in patients under age 40. It is usually associated with a gene mutation called JAK2V617F. The cause of this mutation is unknown.
Breathing difficulty when lying down Dizziness
- Excessive bleeding
- Fullness in the left upper abdomen (due to enlarged spleen)
Headache Itchiness, especially after a warm bath
- Red coloring, especially of the face
Shortness of breath
- Symptoms of
Note: Some of these symptoms are due to increased blood thickness and clotting.
Other symptoms that may occur with this disease:
Bluish skin color Fatigue
- Red skin spots
Signs and tests
The health care provider will perform a physical exam. Tests that may be done include:
Bone marrow biopsy Complete blood countwith differential Comprehensive metabolic panel Erythropoietin level
Genetic test for the JAK2V617F mutation
Oxygen saturation of the blood
Red blood cell mass
Vitamin B12 level
This disease may also affect the results of the following tests:
ESR Lactate dehydrogenase
- Leukocyte alkaline phosphatase
Platelet aggregation test
- Serum uric acid
The goal of treatment is to reduce the thickness of the blood and prevent bleeding and clotting.
A method called phlebotomy is used to decrease blood thickness. One unit of blood (about 1 pint) is removed weekly until the hematocrit level is less than 45 (males) or 42 (females). Then therapy is continued as needed.
Some patients are advised to take aspirin to reduce the risk of blood clots, though it increases the risk for stomach bleeding. Ultraviolet-B light therapy can reduce the severe itching some patients experience.
The disease usually develops slowly. Most patients do not experience any problems related to the disease after being diagnosed. The condition is often diagnosed before severe symptoms occur.
- Acute myelogenous leukemia (
- Bleeding from the stomach or other parts of the intestinal tract
Gout Heart failure Myelofibrosis
- Thrombosis (blood clotting, which can cause a
stroke, heart attack, or other body damage)
Calling your health care provider
Call your health care provider if symptoms of polycythemia vera develop.