Autoimmune hemolytic anemia presenting concurrently with thrombotic thrombocytopenic purpura

Abstract

Thrombotic thrombocytopenic purpura (TTP) is an acute, life-threatening syndrome typically characterized by thrombocytopenia and microangiopathic hemolytic anemia (MAHA). Both genetic and idiopathic TTP are caused by deficient activity of the serine protease ADAMTS-13, allowing its normal substrate, von Willebrand factor, to polymerize and bind platelets under high sheer conditions. Autoimmune TTP is typically treated by plasmapheresis and corticosteroids. Here, we describe a 69-year-old patient presenting with thrombopenia comorbid with "warm" autoimmune hemolytic anemia (WAIHA). Treatment with steroids and plasma exchange initiated at the beginning and patient acquired a clinical as well as biological response. Thrombotic thrombocytopenic purpura (TTP) is a life-threatening condition typically (but not always) associated with autoimmune or genetic inactivation of the protease ADAMTS-13, for which consistently effective therapeutic interventions remain elusive.