Falanga Anna, Rickles Frederick R
Hematology-Oncology Department, Ospedali Riuniti di Bergamo, Largo Barozzi 1, 24128, Bergamo, Italy.
Best Pract Res Clin Haematol. 2003 Sep;16(3):463-82. doi: 10.1016/s1521-6926(03)00059-8.
Life-threatening bleeding, which remains a challenging complication of acute leukaemia, is particularly characteristic of the subtype, acute promyelocytic leukaemia (APL). The clinical picture and laboratory abnormalities are most compatible with the diagnosis of disseminated intravascular coagulation (DIC). Evidence for diffuse activation of the coagulation system, hyperfibrinolysis and systemic elaboration of non-specific protease activity can usually be demonstrated and occurs most commonly during induction chemotherapy. While both host- and tumour-associated mechanisms can be implicated in the pathogenesis of the coagulopathy, leukaemic cell properties appear to be the proximate cause of activation of the haemostatic mechanisms. In this chapter we summarize the current state of knowledge of the pathogenesis of the coagulopathy of APL and the therapeutic approaches that have proved most useful for the management of this complication. Special attention is devoted to the use of all-trans-retinoic acid (ATRA), which has revolutionized the treatment of APL and markedly ameliorated the APL-related coagulopathy.
危及生命的出血仍然是急性白血病的一个具有挑战性的并发症，在急性早幼粒细胞白血病（APL）亚型中尤为典型。临床表现和实验室异常与弥散性血管内凝血（DIC）的诊断最为相符。凝血系统的弥漫性激活、高纤维蛋白溶解以及非特异性蛋白酶活性的全身释放的证据通常可以得到证实，并且最常发生在诱导化疗期间。虽然宿主和肿瘤相关机制都可能与凝血病的发病机制有关，但白血病细胞特性似乎是止血机制激活的直接原因。在本章中，我们总结了目前关于APL凝血病发病机制的知识现状以及已被证明对该并发症管理最有用的治疗方法。特别关注全反式维甲酸（ATRA）的使用，它彻底改变了APL的治疗方式，并显著改善了与APL相关的凝血病。
