Polycythemia Vera

Polycythemia (say: "polly-sigh-thee-me-ah") vera (PV) is a disease in which your body makes too many red blood cells. Polycythemia vera (also known as primary polycythemia, erythema, polycythemia rubra vera, splenomegalic polycythemia, and Vaquez-Osler disease) is a chronic, myeloproliferative disorder. It's characterized by increased red blood cell (RBC) mass, leukocytosis, thrombocytosis, and increased hemoglobin concentration, with normal or decreased plasma volume. It usually occurs between ages 40 and 60, most commonly among men of Jewish ancestry. It seldom affects children or blacks and doesn't appear to be familial.

Causes

In polycythemia vera, uncontrolled and rapid cellular reproduction and maturation cause proliferation or hyperplasia of all bone marrow cells (panmyelosis). The cause of such uncontrolled cellular activity is unknown, but it is probably the result of a multipotential stem cell defect.

Symptoms

The symptoms of polycythemia vera come on slowly. Because there are too many red blood cells, there is more blood in the body than normal, it is thicker than normal and has difficulty circulating in small blood vessels. Symptoms may include:

• poor oxygen circulation - headache, dizziness, vertigo, ringing in the ears (tinnitus), vision changes, chest pain
• abnormal bleeding - nosebleeds, gums bleeding, bruising, digestive system bleeding
• enlarged spleen (splenomegaly) and/or liver (hepatomegaly)
• abnormal blood clots in veins in the body ( thrombosis )
• may develop Budd-Chiari syndrome (obstruction of the main vein of the liver)
• itching (pruritis), especially after a hot bath
• ruddy (reddened) complexion - may be seen in the face, palms, nailbeds, mucous membranes in the mouth, and conjunctiva of the eye

Diagnostic tests

In addition to a complete medical history and physical examination, diagnostic procedures for polycythemia may include additional blood tests to observe the increased number of red blood cells in the body, and distinguish it from other conditions which could cause the red blood cell count to increase (such as with certain cardiac and respiratory diseases, erythremia, and certain tumors).

Treatment

Phlebotomy, the primary treatment, can be performed repeatedly and can reduce RBC mass promptly. It's best used for patients with mild disease and for young patients. The frequency of phlebotomy and the amount of blood removed each time depend on the patient's condition. Typically, 350 to 500 ml of blood can be removed every other day until the patient's hematocrit is reduced to the low-normal range. After repeated phlebotomies, the patient develops iron deficiency, which stabilizes RBC production and reduces the need for phlebotomy. Phlebotomy doesn't reduce the WBC or platelet count and won't control the hyperuricemia associated with marrow cell proliferation.

Myelosuppressive therapy may be used for patients with severe symptoms, such as extreme thrombocytosis, a rapidly enlarging spleen, and hypermetabolism. It's also used for elderly patients who have difficulty tolerating the phlebotomy procedure. Radioactive phosphorus (32P) or chemotherapeutic agents, such as melphalan, busulfan, and chlorambucil, can satisfactorily control the disease in most cases. However, these agents may cause leukemia and should be reserved for older patients and those with serious problems not controlled by phlebotomy. Patients of any age who have had previous thrombotic problems should be considered for myelosuppressive therapy.

Pheresis technology allows removal of RBCs, WBCs, and platelets individually or collectively (and provides these cellular components for blood banks). Pheresis also permits the return of plasma to the patient, thereby diluting the blood and reducing hypovolemic symptoms.

As appropriate, additional treatments include administration of cyproheptadine (12 to 16 mg/day) and allopurinol (300 mg/day) to reduce serum uric acid levels. Treatment usually improves symptomatic splenomegaly; rarely, splenectomy may be performed.

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