Disseminated intravascular coagulation (DIC) associated with hematologic malignancies, particularly acute promyelocytic leukemia (APL), is characterized by marked fibrinolytic activation, which leads to severe bleeding complications. Therefore, appropriate diagnosis and management of DIC are crucial for preventing bleeding-related mortality. However, to date, no clinical guidelines have specifically addressed hematologic malignancy-associated DIC. Therefore, we developed diagnostic and management algorithms for DIC based on a systematic literature review. Notably, these guidelines recommend using the JSTH DIC diagnostic criteria (2017 version) or the former Ministry of Health and Welfare DIC diagnostic criteria (1983 version) to diagnose DIC. Furthermore, in the management of DIC, it is essential to treat the underlying disease through transfusion of platelet concentrates and fresh frozen plasma, if necessary. A systematic review of antifibrinolytic and anticoagulant therapies concluded that tranexamic acid therapy is not strongly recommended for patients with APL undergoing treatment with all-trans retinoic acid (Grade 1C). The use of recombinant thrombomodulin is weakly recommended (Grade 2B), whereas the use of other anticoagulants, including heparin and serine protease inhibitors, is weakly not recommended (Grade 2C). Therefore, we hope that these guidelines will help physicians find the best possible solutions in clinical practice.© 2024. Japanese Society of Hematology.
