Information about the Condition

polycythemia Vera

What do doctors call this condition?

Primary polycythemia

What is this condition?

Polycythemia vera is a chronic bone marrow disorder characterized by increased red blood cell mass, increased white cell production, thrombocytosis, and increased hemoglobin concentration, with normal or increased plasma volume. It usually occurs between ages 40 and 60, most commonly among males of Jewish ancestry. It rarely affects children or Blacks and doesn’t appear to run in families.

The prognosis depends on the person’s age at diagnosis, the treatment used, and complications. The mortality rate is high if the disease is untreated or is associated with leukemia or other types of cancer.

What causes this condition?

In polycythemia vera, uncontrolled and rapid cell reproduction and maturation cause proliferation of all bone marrow cells. The cause of the uncontrolled cellular activity is unknown. Increased red blood cell mass causes blood thickening, which slows blood flow to small blood vessels. These conditions combine with thrombocytosis to promote blockages in some blood vessels.

What are its symptoms?

In its early stages, polycythemia vera usually produces no symptoms . However, as changes in circulation develop, the person may complain of a vague feeling of fUllness in the head, headache, dizziness, and other symptoms, depending on the body system affected. Paradoxically, hemorrhage may occur as a complication of polycythemia vera.

How is it diagnosed?

Lab studies confirm polycythemia vera by showing increased red blood cell mass and other characteristic findings. Bone marrow biopsy (removal and analysis of tissue) reveals increased levels of all bone marrow components.

How is it treated?

Phlebotomy (blood removal therapy) can reduce the red blood cell mass promptly. The frequency of phlebotomy and the amount of blood removed each time depend on the persons condition. Typically, 350 to 500 milliliters of blood can be removed every other day. After repeated phlebotomies, the person develops iron deficiency, which stabilizes red blood cell production and reduces the need for phlebotomy.

For severe symptoms, therapy to depress the bone marrow may be used. In the past, radioactive phosphorus or cancer drugs could usually control the disease. But these agents may cause leukemia and should be reserved for older people and those with problems uncontrolled by phlebotomy. Currently, the preferred myelosuppressive agent is the drug Hydrea. People who’ve had previous problems with blood clot formation should be considered for myelosuppressive therapy