Underlying disorders of disseminated intravascular coagulation: Communication from the ISTH SSC Subcommittees on Disseminated Intravascular Coagulation and Perioperative and Critical Care Thrombosis and Hemostasis

Background: Disseminated intravascular coagulation (DIC), a systemic activation of coagulation, presents with multiple clinical and laboratory manifestations. In this International Society on Thrombosis and Haemostasis (ISTH) communication, we examined the importance of identifying the underlying disorder causing DIC to help physicians in the diagnosis and management of this common and severe condition. Methods: Eight DIC experts participated in a three-step consensus process that searched for published guidelines and diagnostic scores on DIC to create a preliminary list of DIC underlying disorders from those reported in the literature. Overall, 13 papers were identified, including three guidelines, one harmonization paper by the ISTH, one ISTH recommendation paper on cancer-associated DIC, five general diagnostic scores, two scores specific for pregnancy, and one specific for children. We then assessed the strength of the evidence on the association between the disease and DIC as many postulated DIC-associated disorders are rare. Key Results: Eight main subgroups - ‘severe infection’, ‘solid tumour’, ‘haematological neoplasia’, ‘pregnancy complication’, ‘vascular disease’, ‘newborn-complication’, ‘tissue damage due to internal or external insult’, and ‘chemical and biological agent’ - and a detailed list of specific causes of DIC were provided. Conclusions & Inferences: Our results suggest more data are needed to determine the association between DIC and specific diseases such as malignant lymphoma, colorectal cancer, or vasculitis, for which the evidence remains limited. When a patient develops a coagulopathy consistent with DIC, the first step is to immediately search for an underlying disorder, including specific causes that are rarely associated with DIC and to consider that patients may have more than one cause of DIC to identify the principal precipitating disorder to prioritize treatment.