Polycythemia rubra vera.

摘要: PV represents a clonal disorder characterized by excessive erythropoiesis accompanied low serum EPO levels. Two populations of erythroid progenitors have been identified in the BM patients with PV. One population responds normally to and other, malignant clone, is exquisitely sensitive EPO. The latter phenomenon regarded as most readily accepted explanation for pathophysiology this disease, although other mechanisms proposed. Thrombohemorrhagiccomplications, which usually correlate hematocrit level, are important because their frequency severity. Preventing such complications one therapeutic goals. Diagnostic criteria established enabling an accurate diagnosis PV, majority live excess 10 years. Some will develop falling secondary PPMM, also termed spent phase. Patients may acute leukemia, increased incidence if myelosuppressive therapy used, compared phlebotomies alone. initial treatment phlebotomy 45% or less. studies PVSG shown that chlorambucil produces mortality from malignancy, 32P associated superior survivals equivalent. Because substantial risk thrombotic complications, elderly patients, ie, those older than 70 years, should be treated initially therapy, 32P. Hydroxyurea appears less leukemogenic alkylating agents; however, recent reports questioned this. IFN promising new agent become standard form future.