Polycythemia classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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Classification

Primary polycythemia (Polycythemia vera)

Main article: Polycythemia vera

Primary polycythemia, often called polycythemia vera (PCV), polycythemia rubra vera (PRV), or erythremia, occurs when excess red blood cells are produced as a result of an abnormality of the bone marrow. Often, excess white blood cells and platelets are also produced. Polycythemia vera is classified as a myeloproliferative disease.

Secondary polycythemia

Secondary polycythemia is caused by either natural or artificial increases in the production of erythropoietin that result in an increased production of erythrocytes. In secondary polycythemia, there may be 6 to 8 million and occasionally 9 million erythrocytes per cubic millimeter of blood. A type of secondary polycythemia in which the production of erythropoietin increases appropriately is called physiologic polycythemia. Physiologic polycythemia occurs in individuals living at high altitudes, where oxygen availability is less than at sea level. Many athletes train at higher altitudes to take advantage of this effect — a legal form of blood doping. Actual polycythemia sufferers have been known to use their condition as an athletic advantage for greater stamina.

Other causes of secondary polycythemia include smoking, renal or liver tumors, or heart or lung diseases that result in hypoxia. Endocrine abnormalities, prominently including pheochromocytoma and adrenal adenoma with Cushing's syndrome are also secondary causes. Athletes and bodybuilders who abuse anabolic steroids or erythropoietin may develop secondary polycythemia.