Recurrent Hemolytic Uremic Syndrome: Case Report

Dr. M. Meroni, Servizio di Nefrologia e Dialisi, Ospedale di Vimercate, via C. Battisti 23, I-20059 Vimercate (Italy) Dear Sir, Hemolytic-uremic syndrome (HUS) is a disease of infants and children, but this condition may be found in adults too. Females are more commonly affected, and they usually show a history of oral contraception or recent delivery [1]. At present, however, a clear distinction between HUS and thrombotic thrombocytopenic pur-pura is quite difficult. Recurrent attacks of HUS in adults have been seldom recordered, and the factors responsible for the recurrences were not always known [2]. We have observed a 52-year-old female with acute renal failure in course of classic manifestations of HUS: weakness, petechiae, and ecchymoses in the skin, hema-temesis, melena, and hematuria with mild proteinuria. Hemoglobin was 8.8 g/dl, reticulocytes 21%o, white blood cells, 12,000/mm3, platelets 105,000/mm3, plasma fibri-nogen 140 mg/dl, fibrin split products 80 μg/ml, blood urea nitrogen 131 mg/dl, and serum creatinine 6.22 mg/dl. The renal biopsy specimen has been studied by light, immunoñuorescent, and electron microscopy and showed pathognomonic lesions of HUS (fig. 1). The patient was treated with prednisolone pulses, hemodialysis, plasmapheresis and she left the hospital after 42 days. Serum creatinine was 1.36 mg/dl. In coincidence with abrupt withdrawal of prednisolone, she developed a relapse of HUS with slight renal involvement (serum creatinine 1.79 mg/dl, blood urea nitrogen 32 mg/dl), but with severe involvement of skin, intestinal tract, myocardium, and brain. She died after 25 days in spite of prednisolone, plasmapheretic, and vitamin E treatment. At autopsy the pathologic findings were small microthrombi in brain, myocardium, kidney, thyroid, peritoneum, omentum, jejunum, and ileum. Fig. 1. Electron micrograph from a biopsy specimen showing thickening of the capillary wall, effacement of foot processes, wrinkling of the basement membrane (asterisks), widening of the suben-dothelial space, which contains rarefied and granular material of variable electron density