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.