Tefferi A, Solberg L A, Silverstein M N
Division of Hematology and Internal Medicine (AT, MNS), Mayo Clinic and Mayo Foundation, Rochester, Minnesota, USA.
Am J Med. 2000 Aug 1;109(2):141-9. doi: 10.1016/s0002-9343(00)00449-6.
Polycythemia vera and essential thrombocythemia pose specific management issues that distinguish them from other chronic myeloproliferative disorders. They are associated with a better prognosis, as well as a variable risk of thrombohemorrhagic complications. In addition, essential thrombocythemia occurs comparatively more often in young people and women. Treatment strategies for patients with polycythemia vera and essential thrombocythemia must consider the possibility of long-term survival, morbidity from thrombotic complications, transformation into myelofibrosis with myeloid metaplasia or acute myeloid leukemia, and the effect of specific therapies on the incidence of leukemic transformation and on pregnancy. There is increasing concern about the possible leukemogenic effect of hydroxyurea. Newer therapeutic agents, including interferon alpha and anagrelide, are being used more often. Ongoing studies are reexamining the effects of low-dose aspirin in preventing thrombotic complications.
真性红细胞增多症和原发性血小板增多症存在一些特定的管理问题，这些问题将它们与其他慢性骨髓增殖性疾病区分开来。它们的预后较好，但血栓出血并发症的风险各不相同。此外，原发性血小板增多症在年轻人和女性中相对更常见。真性红细胞增多症和原发性血小板增多症患者的治疗策略必须考虑长期生存的可能性、血栓并发症导致的发病率、转化为骨髓纤维化伴髓外化生或急性髓系白血病的情况，以及特定疗法对白血病转化发生率和妊娠的影响。人们越来越关注羟基脲可能的致白血病作用。包括干扰素α和阿那格雷在内的新型治疗药物使用得越来越频繁。正在进行的研究正在重新审视低剂量阿司匹林在预防血栓并发症方面的作用。
