Schafer A I
Blood. 1984 Jul;64(1):1-12.
Bleeding and thrombosis are major causes of morbidity and mortality in patients with myeloproliferative disorders. The significance of uncontrolled polycythemia as a risk factor for thrombosis in these patients has been established. However, the role of thrombocytosis in the pathogenesis of hemostatic complications remains controversial. Abnormalities of platelet function and prolongation of the bleeding time occur in a highly variable number of cases. Specific platelet defects that have been identified in the myeloproliferative defects include abnormal platelet morphology, acquired storage pool disease, platelet membrane abnormalities, and abnormal arachidonic acid metabolism. Causal relationships between any of these specific abnormalities and either bleeding or thrombosis have not been clearly established. The therapeutic efficacy of myelosuppression to reduce the platelet count in patients with thrombocytosis and the role of antiplatelet drugs in the myeloproliferative disorders are controversial issues.
出血和血栓形成是骨髓增殖性疾病患者发病和死亡的主要原因。未控制的红细胞增多症作为这些患者血栓形成的危险因素,其重要性已得到确认。然而,血小板增多症在止血并发症发病机制中的作用仍存在争议。血小板功能异常和出血时间延长在大量病例中表现高度不一。在骨髓增殖性疾病中已确定的特定血小板缺陷包括异常的血小板形态、获得性贮存池病、血小板膜异常和花生四烯酸代谢异常。这些特定异常与出血或血栓形成之间的因果关系尚未明确确立。骨髓抑制降低血小板增多症患者血小板计数的治疗效果以及抗血小板药物在骨髓增殖性疾病中的作用是有争议的问题。