Yamada Shinya, Asakura Hidesaku
Department of Hematology, Kanazawa University Hospital.
Rinsho Ketsueki. 2025;66(8):842-851. doi: 10.11406/rinketsu.66.842.
This paper discusses the diagnostic findings and treatment options for DIC associated with vascular abnormalities based on the "Clinical practice guidelines for management of disseminated intravascular coagulation (DIC) in Japan 2024" released in early 2025. The guidelines define vascular abnormalities as aortic aneurysm, aortic dissection, vasculitis syndromes, and vascular malformations. DIC with enhanced fibrinolysis is a type of DIC often observed in association with vascular abnormalities. Key diagnostic tests for DIC include coagulation activation markers such as thrombin-antithrombin complexes and fibrinolysis activation markers such as plasmin-α plasmin inhibitor complex. Treatment consists of addressing the underlying disease, anticoagulant therapy, and combination therapy with anticoagulants and antifibrinolytics, or potentially observation, depending on bleeding symptoms and test results. Use of Factor XIII concentrates may also be considered. Treatment selection should consider the patient's condition and the treatment duration, with ongoing adjustments based on continuous testing. A well-established testing system and collaboration with experts are crucial in the treatment of DIC associated with vascular abnormalities.
本文基于2025年初发布的《日本2024年弥散性血管内凝血（DIC）管理临床实践指南》，探讨了与血管异常相关的DIC的诊断结果和治疗选择。该指南将血管异常定义为主动脉瘤、主动脉夹层、血管炎综合征和血管畸形。纤维蛋白溶解增强型DIC是一种常与血管异常相关的DIC类型。DIC的关键诊断测试包括凝血激活标志物如凝血酶 - 抗凝血酶复合物，以及纤维蛋白溶解激活标志物如纤溶酶 - α纤溶酶抑制剂复合物。治疗包括治疗基础疾病、抗凝治疗，以及根据出血症状和检测结果，采用抗凝剂和抗纤维蛋白溶解剂联合治疗或可能的观察。也可考虑使用凝血因子 XIII 浓缩剂。治疗选择应考虑患者的病情和治疗持续时间，并根据持续检测进行持续调整。完善的检测系统以及与专家的合作在与血管异常相关的DIC治疗中至关重要。
