Disseminated intravascular coagulation in catastrophic antiphospholipid syndrome: Clinical and hematological characteristics of 23 patients

Background: Disseminated intravascular coagulation (DIC) is an acquired syndrome characterized by formation of microthrombi and fibrin deposition in the microvascular. It is recognized by the presence of thrombocytopenia, elevated fibrin-related markers, prolonged prothrombin time and decreased fibrinogen levels. The catastrophic antiphospholipid syndrome (APS) is characterized by multi-organ thrombosis, mainly involving small vessels. A wide spectrum of disorders may develop DIC features; however, the catastrophic APS has not been previously recognized as a cause of DIC. Objective: To analyze the clinical and laboratory characteristics of catastrophic APS patients with DIC features. Methods: We analyzed the web-site based International Registry of patients with catastrophic APS ("CAPS Registry") (http:// www.med.ub.es/MIMMUN/ FORUM/CAPS.HTM) and select those cases with DIC features. Results: A total of 23/176 (13%) catastrophic APS patients with DIC features were found. The profile of clinical and immunologic characteristics was similar between catastrophic APS patients with and without DIC features. Significant difference was only found in the prevalence of thrombocytopenia (100% in patients with features versus 59 % in patients without these features). Conclusions: DIC features are not rare in catastrophic APS. These findings would support the need for the systematic screening of antiphospholipid antibodies in all patients with DIC features without precipitating factor. Additionally, the existence of DIC features in the context of an APS forces to rule out the presence of the catastrophic variant of this syndrome.