Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome: will recent insight into pathogenesis translate into better treatment? Kelton JG. Transfusion 2002;42(4):388-392.

ABSTRACT:

This editorial comments on some of the recent advances in our understanding of hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) and whether they will translate into therapeutic advances. HUS usually differs from TTP in presentation, natural history and the response of patients to treatment, leading some to conclude that HUS and TTP are different disorders. HUS is more common in children and TTP is more common in adults. A small subset of patients with HUS will have a disorder that is essentially identical to adult TTP. The outcome of HUS in these patients is frequently the same as the outcome for adult TTP, with progressive deterioration and death due to thrombotic events. Approximately 10% of affected children with E. coli progress to HUS and most recover. However, approximately 25% of elderly patients in a nursing home who were reported to have a similar infection developed HUS and/or TTP, and many of these patients died. In terms of treatment, a significant survival advantage is conferred by plasma exchange (PE) compared with plasma infusion. There is some evidence that steroids are useful as well as splenectomy to prevent relapses of TTP. The treatment of both endemic and epidemic HUS continues to be largely supportive, although the author feels that those patients who have severe TTP-like disease benefit from PE therapy.