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| 치료에 반응하지 않은 성인 용혈성 요독 증후군 환자에서 Cryosupernatant를 사용한 혈장 교환 치료 |
| 이규백 , 김윤구 , 이윤하 , 김대중 , 오하영 , 김대원 |
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| Abstract |
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Many patients with Thrombotic Thrombocytopenic PurpuraHemolytic Uremic Syndrome(TTP-HUS) sa- tisfactorily respond to plasma exchange. Some patie- nts, however, respond either not at all or only transiently and incompletely. In the refractory case, endothelial cellderived unusually large von Wille- brand factor multimers(ULvWFM) have an important role in the formation of microthrombi. As the ULvWFM may be removed in the cryoprecipitate, we reason the plasma depleted of cryoprecipitate(the plasma cryosupernatant) should be considered for effectiveness in the treatment of refractory TTP- HUS. We experienced a 48 year old woman presented with diarrhea, jaundice and oliguria. She had mic- roangiopathic hemolytic anemia, renal impairment, platelets of 21,000/mm and LDH 3,258U/L. She had not improved after plasma exchange with fresh frozen plasma(FFP)(1.5 plasma volume x 7 days). On hospital day 8, her HUS had not responded, platelets of 37,000/mm and LDH 1,588U/L Substitution of cryosupematant for FFP was associated with prompt increased in the platelet count to normal and complete resolution of HUS. Therefore. The cryosu- pernatant fraction of plasma should be considered as an alternative to whole FFP for plasma exchange if there is continuing platelet consumption and micro- vascular thrombosis in spite of intensive conven- tional plasma therapy. |
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