Purpura Fulminans Triggered by the Formation of Anti-Endothelial Autoantibodies in a Patient with Chronic Lymphocytic Leukemia

Purpura fulminans (PF), a life-threatening disorder characterized by cutaneous microvascular thrombosis with secondary hemorrhagic infarction, is rare complication of sepsis and may also be caused severe protein C or S deficiency. Here, we describe the case 58-year-old man who developed PF in close association onset chronic lymphocytic leukemia (CLL). initially manifested an ecchymosis right upper leg absence disseminated intravascular coagulation. was likely triggered antiendothelial IgG autoantibodies functionally interfering anticoagulant C-protein S-thrombomodulin system on EA.hy926 endothelial cells modified thrombin generation assay. Although plasma exchange immunosuppressive therapy cyclophosphamide were temporarily effective, eventually progressed patient died from septic shock during treatment-associated neutropenia. In occasions, CLL associated autoimmune-mediated requiring prompt diagnosis aggressive multimodal therapy.