Abstract
It is rare for hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) to occur in members of the same family at the same time. We report two cases of HUS/TTP who were successfully treated with high-dose γ-globulin and gabexate mesilate (FOY®). Case 1 : A 5-year-old girl was admitted to our hospital because of bloody diarrhea. On admission, the peripheral blood analysis revealed leukocytosis without anemia and thrombocytopenia. Gastrointestinal symptoms were improved with antibiotics. Five days later, however, her laboratory tests demonstrated Hb 9.7 g/dl, platelet 29 × 103/μl, WBC 14, 600/μl with 71.0% neutrophils, BUN 30.9 mg/dl and creatinine 0.8 mg/dl. Urine sediment had numerous red cells and the peripheral blood picture revealed marked fragmentation of red cells. Both direct and indirect Coombs tests were negative. Based on the data, a diagnosis of HUS was made. She was treated with gabexate mesilate (FOY®) and high-dose γ-grobulin infusion. The platelet count and BUN were normalized by the 5th day of γ-globulin administration. Case 2 : A 77-year-old male, the grandfather of Case 1, had ileocecal pain when Case 1 was admitted. Shortly after admission, he showed acute abdominal symptoms and underwent surgery. The diagnosis was ischemic enterocolitis. After 8 days, he developed symptoms related to anemia, thrombocytopenia and neurological manifestations, including convulsion and dementia. Initially FOY was administered under the diagnosis of DIC, and the platelet count temporarily increased. Highdose γ-gibulin therapy was started, and the neurological signs and laboratory data were improved significantly. Our experience shows that HUS and TTP might be variable expressions of a single clinical entity and high-dose γ-grobulin therapy could be an effective treatment for HUS/TTP.
