Hemolytic - uremic syndrome successfully treated by iloprost. A case report.

A previously healthy 17-year-man, after fifteen-day-period of flu-like syndrome, was transferred from a nearby general hospital to our institution because of fever, thrombocytopenia, macroematuria, proteinuria and renal failure. A diagnosis of thrombotic microangiopathy (hemolytic - uremic syndrome) was made (table), supported by the finding of fragmented reds cells on peripheral blood smear and by renal biopsy which showed glomerular capillary thrombosis, segmental fibrinoid necrosis, sclerosis and congestion of capillary tufts, some interlobular artery and afferent arterial with subintimal deposits of fibrin, segmental luminal occlusion. The patient had been scored 4 according to the severity score proposed by Rose and Eldor. Therapy was promptly instituted with iloprost continuous infusion at a rate of 1.5 - 2 ng/kg/min for 18 consecutive days, methylprednisone (starting dose 1 g/daily) achieving complete remission (table). He didnʹt receive plasma exchange, fresh frozen plasma or hemodialysis. Thirteen months later, he is still in excellent health, with 1.1 mg/dl serum creatinine. The present case provides evidence that iloprost can be effective in the therapy of thrombotic microangiopathy even in the absence of plasma exchange or plasma infusion.