Kawano Noriaki, Ikezoe Takayuki, Seki Yoshinobu, Yamakawa Kazuma, Okamoto Kohji, Fukatsu Masahiko, Madoiwa Seiji, Uchiyama Toshimasa, Asakura Hidesaku, Yamada Shinya, Koga Shin, Ishikura Hiroyasu, Ito Takashi, Iba Toshiaki, Uchiba Mitsuhiro, Kawasaki Kaoru, Gando Satoshi, Kushimoto Shigeki, Sakamoto Yuichiro, Tamura Toshihisa, Nishio Kenji, Hayakawa Mineji, Matsumoto Takeshi, Mayumi Toshihiko, Wada Hideo
Department of Internal Medicine, Miyazaki Prefectural Miyazaki Hospital, Miyazaki, Japan.
Department of Hematology, Fukushima Medical University School of Medicine, Fukushima, Japan.
Int J Hematol. 2025 May;121(5):605-621. doi: 10.1007/s12185-024-03887-w. Epub 2024 Dec 15.
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.
与血液系统恶性肿瘤相关的弥散性血管内凝血（DIC），尤其是急性早幼粒细胞白血病（APL），其特征是显著的纤溶激活，这会导致严重的出血并发症。因此，对DIC进行恰当的诊断和管理对于预防出血相关的死亡率至关重要。然而，迄今为止，尚无临床指南专门针对血液系统恶性肿瘤相关的DIC。因此，我们基于系统的文献综述制定了DIC的诊断和管理算法。值得注意的是，这些指南推荐使用日本血栓与止血学会（JSTH）DIC诊断标准（2017版）或前卫生福利部DIC诊断标准（1983版）来诊断DIC。此外，在DIC的管理中，如有必要，通过输注血小板浓缩物和新鲜冰冻血浆来治疗基础疾病至关重要。一项关于抗纤溶和抗凝治疗的系统综述得出结论，对于接受全反式维甲酸治疗的APL患者，不强烈推荐使用氨甲环酸治疗（1C级）。弱推荐使用重组血栓调节蛋白（2B级），而不推荐使用包括肝素和丝氨酸蛋白酶抑制剂在内的其他抗凝剂（2C级）。因此，我们希望这些指南将有助于医生在临床实践中找到最佳解决方案。
