Tefferi Ayalew
Division of Hematology, Department of Internal Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
Curr Hematol Rep. 2003 May;2(3):237-41.
The natural history of polycythemia vera (PV) includes an increased lifetime risk of thrombohemorrhagic complications and disease transformation into myelofibrosis with myeloid metaplasia and acute myeloid leukemia. The latter is the primary reason for the shortening of survival that becomes significant after the first decade of disease. Historic nonrandomized studies have suggested that aggressive phlebotomy improves survival in PV. However, prospective randomized studies have failed to demonstrate a better treatment than phlebotomy alone, in terms of survival. However, the addition of cytoreductive therapy to phlebotomy in high-risk patients with PV may reduce the risk of recurrent thrombosis. Other disease features of PV include aquagenic pruritus and microvascular disturbances such as erythromelalgia. This review outlines a practical approach to diagnosis, in addition to treatment of life-threatening and non-life-threatening complications of PV.
真性红细胞增多症（PV）的自然病程包括血栓出血并发症的终生风险增加，以及疾病转化为骨髓纤维化伴髓外化生和急性髓系白血病。后者是疾病第一个十年后生存时间缩短的主要原因。既往非随机研究表明，积极放血可改善PV患者的生存。然而，前瞻性随机研究未能在生存方面证明有比单纯放血更好的治疗方法。不过，在高危PV患者中，放血联合细胞减灭治疗可能降低复发性血栓形成的风险。PV的其他疾病特征包括水诱发性瘙痒和微血管紊乱，如红斑性肢痛症。本综述除了概述PV危及生命和非危及生命并发症的治疗外，还概述了一种实用的诊断方法。
