Plasma Exchange with Cryosupernatant in the Patient with Refractory Hemolytic Uremic Syndrome / 대한신장학회잡지

ABSTRACT

Many patients with Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome(TTP-HUS) satisfactorily respond to plasma exchange. Some patients, however, respond either not at all or only transiently and incompletely. In the refractory case, endothelial cell-derived unusually large von Willebrand 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 microangiopathic hemolytic anemia, renal impairment, platelets of 21,000/mm3 and LDH 3,258U/L. She had not improved after plasma exchange with fresh frozen plasma(FFP)(1.5 plasma volumeX7 days). On hospital day 8, her HUS had not responded, platelets of 37,000/mm3 and LDH 1,588U/L. Substitution of cryosupernatant for FFP was associated with prompt increased in the platelet count to normal and complete resolution of HUS. Therefore. the cryosupernatant fraction of plasma should be considered as an alternative to whole FFP for plasma exchange if there is continuing platelet consumption and microvascular thrombosis in spite of intensive conventional plasma therapy.