Abstract
Thrombotic thrombocytopenia purpura (TTP) caused by a deficiency in ADAMTS-13 activity is considered to involve a subset of thrombotic microangiopathy (TMA). Although concept of TTP is included under the umbrella of TMA, discrimination of TTP from TMA is occasionally difficult in an autoimmune disorder. Herein, we report a case with TTP associated with systemic lupus erythematosus (SLE). In this case, it was difficult to discriminate TTP from TMA and the measurement of ADAMTS-13 activity was useful for obtaining an accurate diagnosis. SLE patients having thrombocytopenia in complication with anemia should be considered a monitoring of ADAMTS-13 activity even though the patients lacked symptoms of TTP related to the microvascular coagulation.
Highlights
Thrombotic thrombocytopenic purpura (TTP) is a lifethreatening syndrome first described by Moschocowitz in 1924 [1]
systemic lupus erythematosus (SLE) patients having thrombocytopenia in complication with anemia should be considered a monitoring of ADAMTS-13 activity even though the patients lacked symptoms of thrombocytopenia purpura (TTP) related to the microvascular coagulation
TTP is clinically characterized by five typical syndromes: thrombocytopenia with platelet consumption, hemolytic anemia characterized by schistocytes, renal impairment, neurological abnormalities, and fever
Summary
Thrombotic thrombocytopenic purpura (TTP) is a lifethreatening syndrome first described by Moschocowitz in 1924 [1]. Besides TTP, microthrombosis without a deficiency in ADAMTS-13 activity can be associated with other diseases such as hemolytic uremic syndrome (HUS), various autoimmune diseases, cytotoxic drugs, human immunodeficiency virus (HIV), malignancies, disseminating intravascular coagulopathy (DIC), and pre-eclampsia [7]. These pathological conditions characterized by microthrombosis including TTP, have been classified as thrombotic microangiopathies (TMA). HBs Ag difficulty of TTP associated with autoimmune diseases such as SLE and the diagnostic efficacy of immediate monitoring of ADAMTS-13 activity for distinguishing TTP from other TMA
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