对弥漫性血管内凝血（DIC）的两种表型进行了系统回顾。DIC分为血栓性和纤溶性表型，其特征分别为血栓形成和出血。DIC与血栓性表型的主要病理是凝血的激活、抗凝能力不足、内皮损伤以及纤溶抑制剂-1（PAI-1）介导的纤溶抑制，导致微血管纤维栓塞和器官功能障碍。DIC与纤溶性表型定义为伴有明显血栓生成的大量凝血酶生成，与基本疾病引起的全身病理性高纤溶血症结合在一起，并由于过度的纤溶产物生成导致严重出血。三个主要的全身病理性高纤溶血症的病因机制包括：i）缺氧内皮细胞和丰富组织型纤溶酶原活化物（t-PA）贮存池中组织型纤溶酶原激活物（t-PA）释放加速；ii）癌细胞和内皮细胞上表达的特定蛋白质和受体增强纤溶酶原向纤溶酶的转化；和iii）替代性纤溶途径。通过DIC的诊断，即可诊断出伴随全身病理性高纤溶血症的DIC。诊断全身病理性高纤溶血症的重要指标包括纤维蛋白原水平的降低、纤维蛋白原和纤维蛋白降解产物（FDP）的升高以及FDP/D二聚体比值。目前，关于DIC纤溶性表型的循证治疗策略的证据不足。止血纤酸（Tranexamic acid）似乎是治疗全身病理性高纤溶血症的少数有效方法之一。对于DIC纤溶性表型的病理机制的阐明、诊断标准的制定以及治疗策略的建立，是血栓形成和止血领域亟待解决的问题。The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).

Two phenotypes of disseminated intravascular coagulation (DIC) are systematically reviewed. DIC is classified into thrombotic and fibrinolytic phenotypes characterized by thrombosis and hemorrhage, respectively. Major pathology of DIC with thrombotic phenotype is the activation of coagulation, insufficient anticoagulation with endothelial injury, and plasminogen activator inhibitor-1 (PAI-1)-mediated inhibition of fibrinolysis, leading to microvascular fibrin thrombosis and organ dysfunction. DIC with fibrinolytic phenotype is defined as massive thrombin generation commonly observed in any type of DIC, combined with systemic pathologic hyperfibrinogenolysis caused by underlying disorder that results in severe bleeding due to excessive plasmin formation. Three major pathomechanisms of systemic hyperfibrinogenolysis have been considered: i) acceleration of tissue-type plasminogen activator (t-PA) release from hypoxic endothelial cells and t-PA rich storage pools, ii) enhancement of the conversion of plasminogen to plasmin due to specific proteins and receptors that are expressed on cancer cells and endothelial cells, and iii) alternative pathways of fibrinolysis. DIC with fibrinolytic phenotype can be diagnosed by DIC diagnosis followed by the recognition of systemic pathologic hyperfibrin(ogen)olysis. Low fibrinogen levels, high fibrinogen and fibrin degradation products (FDP) and the FDP/D-dimer ratio are important for the diagnosis of systemic pathologic hyperfibrin(ogen)olysis. Currently, evidence-based treatment strategies for DIC with fibrinolytic phenotypes are lacking. Tranexamic acid appears to be one of the few methods to be effective in the treatment of systemic pathologic hyperfibrin(ogen)olysis. International cooperation for the elucidation of pathomechanisms, establishment of diagnostic criteria, and treatment strategies for DIC with fibrinolytic phenotype are urgent issues in the field of thrombosis and hemostasis.The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).