Sabljic Nikica, Thachil Jecko, Pantic Nikola, Mitrovic Mirjana
Clinic of Hematology, University Clinical Center of Serbia, Belgrade, Serbia.
Department of Hematology, Manchester Royal Infirmary, Manchester, United Kingdom.
Res Pract Thromb Haemost. 2024 Jul 2;8(5):102499. doi: 10.1016/j.rpth.2024.102499. eCollection 2024 Jul.
Coagulopathy continues to be a major challenge in the management of patients with acute promyelocytic leukemia (APL). Novel differentiating agents have led to improved survival in these patients, but perturbations in coagulation continue to have an impact on their prognosis. The most worrisome of coagulation disturbances is bleeding, which is not an uncommon cause of early death in APL. Despite this, there are no consistent predictors of this high risk of fatal hemorrhage in APL. In this context, the fibrinolytic system has been identified as a crucial role player in APL coagulopathy. However, the current guidelines for the management of APL give little regard to tests that measure the fibrinolytic system while giving more importance to close monitoring of conventional coagulation tests and platelet counts to identify the coagulopathy. More recently, viscoelastic tests have come to usefulness in determining global hemostasis and have been widely used for "diagnosing" hyperfibrinolysis in selected clinical settings. In this review, we attempt to describe risk assessment models for diagnosing APL coagulopathy, describe the possible application of viscoelastic tests in this setting, and persuade clinicians to reconsider the use of antifibrinolytics to improve survival of APL patients.
凝血功能障碍仍然是急性早幼粒细胞白血病（APL）患者治疗中的一项重大挑战。新型分化诱导剂已使这些患者的生存率得到提高，但凝血功能紊乱仍对其预后产生影响。最令人担忧的凝血功能障碍是出血，这是APL患者早期死亡的常见原因之一。尽管如此，目前尚无一致的指标可预测APL患者发生致命性出血的高风险。在此背景下，纤维蛋白溶解系统已被确定为APL凝血功能障碍的关键因素。然而，当前的APL治疗指南很少关注检测纤维蛋白溶解系统的指标，而是更重视密切监测传统凝血试验和血小板计数以识别凝血功能障碍。最近，黏弹性试验已被用于确定整体止血功能，并已在特定临床环境中广泛用于“诊断”纤维蛋白溶解亢进。在这篇综述中，我们试图描述诊断APL凝血功能障碍的风险评估模型，阐述黏弹性试验在该情况下的可能应用，并说服临床医生重新考虑使用抗纤溶药物以提高APL患者的生存率。
