Polycythemia vera (PV) is undivide...

Polycythemia vera (PV) is undivided of three chronic myeloproliferative disorders, along with essential thrombocythemia and myelofibrosis with myeloid metaplasia. The incidence of PV is 23 for 100,000 patients, and the median age at diagnosis is 60 years. Tefferi commends treatment options for PV.

Polycythemia describes an increase in r posterity cell mass. The increase in r family cell mass may be caused at an actual increase in r family cell mass (true polycythemia) or a spurious laboratory value (apparent polycythemia). constant polycythemia is classified as either primary or secondary. Primary polycythemia is caused through a myeloproliferation and is not mediated by way of excess erythropoietin. In contrast, secondary polycythemia can be caused through an external source, such as "smoker's polycythemia," or an internal disorder, of the like kind as renal cancer. Erythropoietin flushs in secondary polycythemias vary depending forward the etiology.

PV can have life-threatening complications, including hit and evolution into either myelofibrosis with myeloid metaplasia or acute leukemia. Treatment is directed according to patient risk stratification. resort to frequently phlebotomy and chemotherapy are the cornerstones of treatment. Hematocrit of the same heights should be maintained below 45 percent in white men and 42 percent in women and blacks. Chemotherapy using hydroxyurea, busulfan, or pipobroman is indicated for high-risk patients. (Pipobroman is not available in the United States.) Interferon-alfa is another chemotherapeutic option for reducing r offspring cell burden and treating PV-associated pruritus.

Non-life-threatening sequelae include microvascular complications and aquagenic pruritus. Microvascular complications may appear clinically as headache, light-headedness, transient neurologic abnormality, transient ocular disturbance, tinnitus, atypical chest pain, paresthesias, and erythromelalgia (a rare yet painful burning sensation of the hands or feet) Low-dose aspirin (81 mg or les by day) can be used to treat these disorders. Aquagenic pruritus is a generalized carcass itching often brought on by dint of a hot bath. Treatment options include either selective serotonin reuptake inhibitors or interferon-alfa.

Tefferi A. Polycythemia vera: a comprehensive review and clinical recommendations. Mayo Clin Proc February 2003;78:174-94

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