Pabinger-Fasching I
Abteilung für Hämatologie und Blutgerinnung, I. Medizinischen Universitätsklinik Wien.
Wien Med Wochenschr. 1991;141(9-10):213-6.
Bleeding and septicemia are the most common causes of death in patients with acute leukemia. Additionally to thrombopenia, which is present in most patients, in part of the patients severe coagulation abnormalities are observed. The prevalence of severe coagulation abnormalities in patients with acute myeloid leukemia including all FAB types is around 12%, in patients with promyelocytic leukemia the prevalence is more than 50%. The etiology of this coagulation abnormality remains unclear. There are 3 mechanisms currently under discussion: activation of coagulation, activation of fibrinolysis and an increased activity of specific leucocyte proteases. In particular, in promyelocytic leukemia the risk of cerebral bleeding is high (10 to 15% of early deaths). The most important therapeutic consequence is an intensified thrombocyte replacement regimen. Additional therapeutic efforts, like administration of heparin or antifibrinolytic drugs, have failed to improve the prognosis of patients with severe coagulation abnormalities.
出血和败血症是急性白血病患者最常见的死亡原因。除大多数患者存在的血小板减少外,部分患者还观察到严重的凝血异常。包括所有FAB类型在内的急性髓系白血病患者中严重凝血异常的发生率约为12%,早幼粒细胞白血病患者的发生率超过50%。这种凝血异常的病因仍不清楚。目前有3种机制正在讨论中:凝血激活、纤溶激活和特定白细胞蛋白酶活性增加。特别是,早幼粒细胞白血病患者发生脑出血的风险很高(早期死亡的10%至15%)。最重要的治疗结果是强化血小板替代方案。其他治疗措施,如使用肝素或抗纤溶药物,未能改善严重凝血异常患者的预后。