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The mainstay of treatment for PV is phlebotomy, which is aimed at reducing hyperviscosity by decreasing the venous hematocrit level to less than 45 percent (0.45) in white men and 42 percent (0.42) in blacks and women.1,14,18 The PVSG reported the best median survival, 12.6 years, for this type of treatment.14 Some features of using phlebotomy alone are attractive, primarily because it is a simple procedure without many risks, except for the eventual development of iron deficiency.8 Some experts have cast doubt on the PVSG findings regarding median survival, noting that up to 50 percent of patients treated with phlebotomy alone had to switch to other treatments by the fifth year.19 The PVSG found a statistically significant increase in the number of thrombotic events within the first three years of initiating treatment, compared with the use of myelosuppressive agents.14 After this period, however, the rate of thrombosis remained the same for both treatment approaches. Immediate, unlimited access to all AFP content. 3d ed. Diagnosis and treatment of polycythemia vera and possible future study designs of the PVSG. Proliferation of mature megakaryocytes that vary in size and shape. Interpretation of measured red cell mass and plasma volume in adults: Expert Panel on Radionuclides of the International Council for Standardization in Haematology. Cuttner J, 11. 1993;270:2218–21. Silver RT. A diagnosis of polycythemia vera (PV) requires the presence of either all 3 major criteria, or the first 2 major criteria plus the minor criterion. Therapeutic recommendations for polycythemia vera. Polycythemia vera. et al. Semin Oncol. A myelosuppressive agent with supplemental phlebotomy is reasonable in this group. Blood. Diagnostic criteria and prognosis in polycythemia vera and essential thrombocythemia. : Proposed criteria for the diagnosis of post-polycythemia vera and post-essential thrombocythemia myelofibrosis: a consensus statement from the International Working Group for Myelofibrosis Research and Treatment. All rights Reserved. Donovan PB, … Rain JD, Fuchtman SM, Individuals with PV usually have very low levels of erythropoietin. He received his medical degree from Saint Louis University School of Medicine, St. Louis, and completed his residency in family medicine at Naval Hospital Jacksonville, Jacksonville, Fla.... ANTHONY J. VIERA, LCDR, MC, USNR, is a staff family physician at Naval Hospital Jacksonville, and assistant professor of family medicine at the Uniformed Services University of the Health Sciences F. Edward Hébert School of Medicine, Bethesda, Md. No single treatment is available for PV. Barosi G, Mesa R, Thiele J, et al. Copyright © 2004 by the American Academy of Family Physicians. The most recent update to the … Polycythemia Vera. In: Wasserman LR, Berk PD, Berlin NI, eds. Diagnosis is made using criteria developed by the Polycythemia Vera Study Group; major criteria include elevated red blood cell mass, normal oxygen saturation, and palpable splenomegaly. Finazzi G, Because polycythemia vera (PV) is a rare type of blood cancer, a diagnosis often comes when you see your doctor for other reasons. Berger U, Br J Haemat. Chlorambucil, busulfan, and pipobroman, all alkylating agents, have fallen out of favor because of concerns about rates of iatrogenic leukemia.19 The agent 32P remains in use with supplemental phlebotomy and has a reported median survival similar to that of phlebotomy alone—10.9 years according to PVSG data14 and 11.8 years according to GISP.20 The myelosuppressive drugs such as 32P had an initial advantage over phlebotomy alone regarding thrombosis rates during the first three years of treatment. Chinn S, Because PV is a rare condition, it has been difficult to assemble patients for well-designed, randomized controlled trials with long- term follow-up. Sources of funding: none reported. Aquagenic pruritus may precede a diagnosis of polycythemia vera by several years or more. Finazzi G. The Leukemia & Lymphoma Society® (LLS) is a global leader in the fight against cancer. Therapeutic options for essential thrombocythemia and polycythemia vera. For more information about bone marrow tests and other lab tests, please see the free LLS publication Understanding Lab and Imaging Tests. Comparison of erythroid progenitor cell growth in vitro in polycythemia vera and chronic myelogenous leukemia: only polycythemia vera has endogenous colonies. 2000;79:103–9. This group’s generally shorter life expectancy lessens the threat of eventual iatrogenic malignancy. Thrombosis accounts for the missing item, see the free LLS publication Understanding lab and Imaging tests is in... Percent of patients with elevated hemoglobin or hematocrit levels, splenomegaly, or transformation to a aggressive..., molecular testing for the missing item, see the free AFP email Table of contents with... The production of new therapeutic agents have been developed, these criteria remain standard. Copyright © 2004 by the American Academy of Family physicians condition that ca n't be cured 4. 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