Department of Thrombosis and Vascular Biology, Guy's and St. Thomas' NHS Foundation Trust, London, UK.
Br J Haematol. 2012 Jan;156(1):24-36. doi: 10.1111/j.1365-2141.2011.08922.x. Epub 2011 Nov 3.
Coagulopathy occurs in most patients with (APML) and is life-threatening; therefore prompt diagnosis and recognition of any coagulation defect is imperative. Unfortunately haemorrhage remains a major cause of early death, preventing some from reaching treatment. The coagulopathy is caused directly or indirectly by the leukaemic cells through expression of activators of coagulation and fibrinolysis, proteases and cytokine generation, compounded by failure of platelet production due to marrow invasion. At presentation the predominant feature is usually hyperfibrinolysis. Since the introduction of all-trans retinoic acid (ATRA), patient outcome has dramatically improved; yet, haemorrhagic complications remain the most frequent cause of mortality. Thrombotic complications occur but are less well recognized and potentially underreported. Supportive measures and prompt initiation of ATRA currently represent the mainstay of treatment of the coagulopathy in patients with suspected APML, but unanswered questions remain as to the optimal approach to further decrease the associated haemorrhagic and thrombotic risks. In particular, it is unclear how to best predict and monitor the coagulopathy; whether there is a role for the early use of antifibrinolytics; the most appropriate trigger for giving fibrinogen replacement and the value of low-dose anticoagulation to suppress coagulation activation once fibrinolysis has been suppressed.
大多数（APML）患者都会发生凝血功能障碍，且具有生命威胁；因此，及时诊断和识别任何凝血功能障碍至关重要。不幸的是，出血仍然是早期死亡的主要原因，使一些患者无法接受治疗。凝血功能障碍是由白血病细胞通过表达凝血和纤维蛋白溶解激活物、蛋白酶和细胞因子生成直接或间接引起的，同时由于骨髓浸润导致血小板生成失败而加剧。在发病时，主要特征通常是过度纤维蛋白溶解。自从全反式维甲酸（ATRA）问世以来，患者的预后已显著改善；然而，出血并发症仍然是导致死亡率的最常见原因。血栓并发症虽然较少见，但尚未得到充分认识，且可能报告不足。支持性措施和及时开始使用 ATRA 目前是治疗疑似 APML 患者凝血功能障碍的主要方法，但仍存在一些尚未解决的问题，例如如何进一步降低相关出血和血栓风险的最佳方法。特别是，目前尚不清楚如何最好地预测和监测凝血功能障碍；是否需要早期使用纤维蛋白溶解抑制剂；给予纤维蛋白原替代物的最佳触发因素以及低剂量抗凝抑制纤维蛋白溶解后的凝血激活的价值。
