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

Abstract

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