A Clinical Study on Adult Thrombotic Thrombocytopenic Purpura / 대한신장학회잡지

ABSTRACT

OBJECTIVE: Thrombotic thrombocytopenic purpura (TTP) is a disorder characterized by microangiopathic hemolytic anemia, thrombocytopenia, fluctuating neurological symptoms and a variable degree of impairment of renal function, which are thought to be due to endothelial cell injury, platelet activation and subsequent formation of thrombi in the microcirculaion. It usually occurs in adults but few reports are available on etiology, clinical manifestation, progression and the outcome of disease in Korea. METHODS: We investigated 10 adult patients who had admitted and were diagnosed as TTP in Department of Internal Medicine, Chungnam National University Hospital from Dec. 1994 to Jul. 2001. RESULTS: The male to female ratio was 1 4. The etiologic antecedants were infectious diarrhea in 3 patients, upper respiratory infection in 3 patients and pregnancy was related with TTP in 2 patents. The clinical manifestations were variable neurologic symptoms(100%), renal involvement(90%), hemorrhagic manifestations(80%), fever(60%), and diarrhea(40%). Acute renal failure was noted in 6 patients and hemodialysis was required in 5 patients. Plasma exchange was performed in 9 patients and corticosteroid was prescribed in 8 patients, simultaneously. Vincristine, azatioprine and cyclophosphamide were added in 2 patients. One patient died of hepatic failure. Seven patients showed complete recovery. One patient progressed to end-stage renal failure. The other patient showed multiple recurrences. CONCLUSION: It is thought that a considerable number of TTP patients show very serious acute renal failure and plasma exchange should be included in therapeutic modalities of TTP.