Finazzi Guido
Department of Hematology, Ospedali Riuniti di Bergamo, Bergamo, Italy.
Semin Thromb Hemost. 2006 Apr;32(3):276-82. doi: 10.1055/s-2006-939439.
The clinical course of polycythemia vera (PV) is marked by a high incidence of thrombotic complications, which represent the main cause of morbidity and mortality. Major predictors of vascular events are increasing age and previous thrombosis. Myelosuppressive drugs can reduce the rate of thrombosis, but there is concern that their use increases the risk of transformation into acute leukemia. To tackle this dilemma, a risk-oriented management strategy is recommended. Low-risk patients should be treated with phlebotomy and low-dose aspirin based on the results of the European Collaboration on Low-Dose Aspirin in Polycythemia study. Cytotoxic therapy is indicated in high-risk patients, and the drug of choice is hydroxyurea because its leukemogenicity is low. New therapeutic options, that theoretically are devoid of leukemic risk (such as interferon alpha and imatinib), should be reserved for selected patients and require additional clinical experience.
真性红细胞增多症（PV）的临床病程以血栓形成并发症的高发生率为特征，这些并发症是发病和死亡的主要原因。血管事件的主要预测因素是年龄增长和既往血栓形成。骨髓抑制药物可降低血栓形成率，但人们担心使用这些药物会增加转化为急性白血病的风险。为解决这一困境，建议采用以风险为导向的管理策略。根据欧洲低剂量阿司匹林治疗真性红细胞增多症协作研究的结果，低风险患者应采用放血疗法和低剂量阿司匹林进行治疗。高风险患者应采用细胞毒性疗法，首选药物是羟基脲，因为其致白血病性较低。理论上无白血病风险的新治疗选择（如干扰素α和伊马替尼）应保留给特定患者，且需要更多临床经验。
