A case report of adult hemolytic uremic syndrome

Hemolytic uremic syndrome (HUS) is a rare disease in adults, however, in childhood the syndrome is not rarely found and shows usually a benign outcome. Most cases of HUS in adults show a resistance for any therapeutic devices such as anticoagulants or antiplatelet therapy. Maintenance hemodialysis therapy is usually necessary because of the persistance of chronic renal failure. Recently some papers which demonstrated an efficacy of plasmapheresis (PP) therapy for the syndrome, were reported. We report a case of adult HUS treated with PP and antiplatelet therapy. A 37-year-old male patient was admitted for nausea, peripheral edema and oliguria. Seven days prior to the admission he complainted of teethache and headache and received a prescription of antiinflammatory drugs (SEDES®, NOUSHIN®) and an antibiotic (ACROMYCIN®) On admission hemolytic anemia and thrombocytopenia were pointed out in addition to acute renal failure. RBC counts, hemoglobin, hematocrit and platelet counts were 150×104/cmm, 5.1 g/100 ml, 13.8% and 98, 000/cmm, respectively. Some RBCs showed a deformity such as burr cells and helmet cells. Serum LDH lever was 5535 IU/1, BUN, creatinine and potassium concentrations were 201, 21.1 mg/100ml and 7.1 mEq/l. A renal histology done on the 13th day after admission showed thrombi in some interlobular arteries and glomerular capillaries and some glomeruli showed ischemic changes. These findings were compatible with renal changes in HUS. PP therapy was carried out twice using 3, 000 ml of fresh frozen plasma besides blood transfusion and hemodialysis therapy. His renal function was gradually improved, and anemia and thrombocytopenia were also recovered. After a mandibular biopsy which disclosed ameloblastoma, a decrease of urine output, azotemia and anemia appeared again and peripheral platelet counts also decreased, A third PP therapy was carried out, which brought about a recovery of thrombocytopenia. But anemia and renal failure were not recovered and repeated hemodialysis therapy was continued. The recurrence of hemolytic uremic episode following a surgical manipulation to the mandibular ameloblastoma is to suggest that the tumor might have some relation to an etiology of the syndrome, because the syndrome occurred after teethache in the patient. We think PP therapy was effective for the syndrome by means of a removal of some humoral substances released from the tumor which might cccur hemolytic uremic episode.