Introduction

Thrombotic thrombocytopenic purpura (TTP) is a rare and potentially fatal hematologic disease. Fifty years ago, before the era of effective treatment, the diagnosis of TTP was based on the progressive appearance of the “pentad” of clinical manifestations: microangiopathic hemolytic anemia, thrombocytopenia, renal and neurological abnormalities, and fever 1. Recent studies have demonstrated that deficiency in the von Willebrand factor (vWF) cleaving protease ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin motifs 13) causes TTP. The deficiency of ADAMTS13 can be genetic or more common, acquired, resulting from autoimmune production of inhibitory anti-ADAMTS13 antibodies 2.
PEX (plasma exchange) has now become the cornerstone of the management of TTP. Timely, extensive PEX has been indicated to reduce the mortality rate to <10%, resulting in >90% short-term effectiveness. However, few attentions have been paid to the complication of PEX since PEX requires insertion of a central venous dialysis catheter, with its risk for hemorrhage, thrombosis and infection 3.
In this case, we describe a young male who was firstly defined by either simultaneous or sequential combination of immune thrombocytopenia and autoimmune hemolytic anemia with a positive direct anti globulin test (DAT) in the absence of known underlying etiology and diagnosed as TTP. He responded well to the timely PEX but suffered catheter-associated thrombosis.