Sepsis-induced disseminated intravascular coagulation with features of thrombotic thrombocytopenic purpura: a fatal fulminant syndrome.

Disseminated intravascular coagulation (DIC) and thrombotic thrombocytopenic purpura (TTP) are different disease states, while ADAMTS13 deficiency could occur in sepsis-induced DIC. We report 2 patients who had septic DIC with features of idiopathic TTP characterized by low ADAMTS13 activity and positive ADAMTS13 inhibitor. They had a specific fulminant course and fatal outcome, which might represent a new specific syndrome.