Barbui Tiziano, Finazzi Guido
Department of Hematology-Oncology, Ospedali Riuniti, Bergamo, Italy.
Semin Thromb Hemost. 2007 Jun;33(4):321-9. doi: 10.1055/s-2007-976166.
The clinical course of polycythemia vera (PV) and essential thrombocythemia (ET) is characterized by an increased incidence of thrombotic and hemorrhagic complications and an inherent tendency to progress into myelofibrosis or acute myeloid leukemia. Major predictors of vascular events are increasing age and previous thrombosis. Myelosuppressive drugs can reduce the rate of thromboses and hemorrhages, but there is concern that their use accelerates the rate of leukemic transformation. Thus, a risk-oriented management strategy is recommended. Low-risk patients with PV should be treated with phlebotomy and low-dose aspirin, whereas those with ET can be left untreated. Cytotoxic agents are recommended in high-risk cases and hydroxyurea is the drug of choice in most patients. Interferon alpha or anagrelide could be considered in selected young patients or as second-line therapy in those refractory or intolerant of hydroxyurea. The recent identification of JAK2V617F mutation in a substantial proportion of patients with PV and ET raises new questions regarding both risk classification and management, but additional studies on these issues are required.
真性红细胞增多症（PV）和原发性血小板增多症（ET）的临床病程特点为血栓形成和出血并发症的发生率增加，以及向骨髓纤维化或急性髓系白血病进展的内在倾向。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和出血的发生率，但人们担心其使用会加速白血病转化的速度。因此，建议采用以风险为导向的管理策略。低风险PV患者应采用放血疗法和小剂量阿司匹林治疗，而ET患者可不予治疗。高风险病例建议使用细胞毒性药物，大多数患者首选羟基脲。对于部分年轻患者或对羟基脲难治或不耐受的患者，可考虑使用干扰素α或阿那格雷作为二线治疗。最近在相当一部分PV和ET患者中发现JAK2V617F突变，这就风险分类和管理方面提出了新问题，但需要对这些问题进行更多研究。
