Thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. Baker KR, Moake JL. Current Opinion in Pediatrics 2000;12(1):23-28.

This paper presents an overview of the diagnosis, pathophysiology, and therapy for thrombotic thrombocytopenic purpura (TTP) and the hemolytic-uremic syndrome (HUS). Most relevant is the review of therapeutic TTP studies. Some of the findings indicate that neither the number of plasma volumes exchanged per procedure nor the use of various taper protocols in the use of plasma exchange after remission influences the rate (40%) of subsequent TTP relapses; that plasma treated with solvent and detergent is as effective as untreated plasma for TTP; and that along with solvent or detergent-treated plasma, cryosupernatant is sometimes effective in refractory TTP. Treatment of acquired HUS, on the other hand, is controversial. Supportive care, including dialysis and renal transplantation, if necessary, is universally recommended, but few randomized trials have evaluated other treatments. It is unclear whether antibiotic therapy is dangerous. A study testing the efficacy of prednisone confirmed, however, that corticosteroids do not alter the number of seizures or the need for blood transfusion or dialysis during the acute phase of acquired HUS. Testing of experimental vaccines is underway in animals that may lead to studies in children.