Abstract:

A 47-year-old man with abrupt onset trilogy manifesting thrombocytopenia and microangiopathic hemolytic anemia and acute renal failure, accompanying with massive proteinuria, microscopic hematuria, low serum complement C3 and extreme deficiency of ADAMTS13 activity, was proved as thrombotic microangiopathy (TMA) by renal biopsy pathology. Although large amount plasma exchange and methylprednisolone pulse therapy was administrated, the hemolysis anemia was remission, but the TTP relapsed repeatedly and the renal function deteriorated progressively. Eight months later, he developed to ESRD, and was treated with maintenance hemodialysis.

Key words: thrombotic microangiopathy , ,thrombotic thrombocytopenic purpura, ADAMTS 13 , ,atypical hemolytic uremic syndrome