Falanga A, Barbui T
Hematology-Oncology Department, Ospedali Riuniti, Largo Barozzi n.1, I-24128 Bergamo, Italy.
Acta Haematol. 2001;106(1-2):43-51. doi: 10.1159/000046588.
Life-threatening bleeding is frequent in acute leukemias, particularly in acute promyelocytic leukemia (APL), a distinct subtype of acute myelogenous leukemia, characterized by the balanced reciprocal translocation between chromosomes 15 and 17. Laboratory assessments show profound hemostatic imbalance compatible with the clinical picture of disseminated intravascular coagulation. Activation of the coagulation system, hyperfibrinolysis and nonspecific proteases activity can be observed in this condition. An important pathogenetic role is attributed to the leukemic cell properties for activating hemostatic mechanisms. This review will summarize what is currently known about the coagulopathy of APL, the principal pathogenetic mechanisms, and the therapeutic tools for the management of this complication. Special attention will be devoted to the new therapy with all-trans retinoic acid, which has completely changed the natural history of APL and APL-related coagulopathy.
危及生命的出血在急性白血病中很常见，尤其是在急性早幼粒细胞白血病（APL）中，它是急性髓系白血病的一个独特亚型，其特征是15号和17号染色体之间的平衡易位。实验室评估显示存在与弥散性血管内凝血临床表现相符的严重止血失衡。在此情况下可观察到凝血系统激活、高纤维蛋白溶解和非特异性蛋白酶活性。白血病细胞激活止血机制的特性被认为具有重要的发病机制作用。本综述将总结目前关于APL凝血病、主要发病机制以及该并发症管理的治疗工具的已知信息。将特别关注全反式维甲酸的新疗法，它彻底改变了APL及APL相关凝血病的自然病程。
