F Keller1, H Schwarze, A Schwarz. 1. Sektion Nephrologie, Medizinische Universitätsklinik, Ulm, Bundesrepublik, Deutschland.
Abstract
BACKGROUND: According to recent research, the hemolytic-uremic syndrome (HUS) and thrombotic-thrombocytopenic purpura (TTP) are variable expressions of the same entity (HUS-TTP) with a common pathomechanism (endothelial cell damage, microthrombi) and common treatment (plasma infusion, plasmapheresis). The condition is still serious with a poor prognosis, and the therapeutic regimen is not yet standardized (cryosupernatant and factor VIII free plasma, steroids, immunoglobulins, anticoagulation, dextrane, prostacyclin, vincristine, splenectomy?). CLINICAL OBSERVATIONS AND REVIEW OF THE LITERATURE: Over an observation period of 15 years we considered the differential diagnosis of HUS-TTP in 34 patients, and treated 11 patients with 12 clinical courses specifically with fresh-frozen plasma (plasmapheresis was additionally performed in 10 of them). The 12 courses were retrospectively evaluated and compared with results achieved in the literature. The mean age of the patients was 43 years (+/- 14), and 9 of the 11 patients were women (2 courses given to one woman). The hemolysis improved in 9 of 12 courses, the cerebral manifestation in 3 of 4 cases, and the thrombocytopenia in 2 of 4 cases. Renal failure responded in only 4 of 9 cases and the response was delayed in these patients. Three patients died: one of brain edema due to TTP-specific cerebral microangiopathy and two due to the underlying disease (lupus erythematosus, mixed connective tissue disease). CONCLUSION: Treatment of HUS-TTP is started with fresh-frozen plasma infusions (1-1.5 liters/day), but plasmapheresis should be added 2 days later (3 x 4 liters/week, whereby 2 liters should be given as fresh-frozen plasma). The administration of fresh-frozen plasma must be continued every day. In resistant cases, specific therapy should not be terminated before 4 weeks.
BACKGROUND: According to recent research, the hemolytic-uremic syndrome (HUS) and thrombotic-thrombocytopenic purpura (TTP) are variable expressions of the same entity (HUS-TTP) with a common pathomechanism (endothelial cell damage, microthrombi) and common treatment (plasma infusion, plasmapheresis). The condition is still serious with a poor prognosis, and the therapeutic regimen is not yet standardized (cryosupernatant and factor VIII free plasma, steroids, immunoglobulins, anticoagulation, dextrane, prostacyclin, vincristine, splenectomy?). CLINICAL OBSERVATIONS AND REVIEW OF THE LITERATURE: Over an observation period of 15 years we considered the differential diagnosis of HUS-TTP in 34 patients, and treated 11 patients with 12 clinical courses specifically with fresh-frozen plasma (plasmapheresis was additionally performed in 10 of them). The 12 courses were retrospectively evaluated and compared with results achieved in the literature. The mean age of the patients was 43 years (+/- 14), and 9 of the 11 patients were women (2 courses given to one woman). The hemolysis improved in 9 of 12 courses, the cerebral manifestation in 3 of 4 cases, and the thrombocytopenia in 2 of 4 cases. Renal failure responded in only 4 of 9 cases and the response was delayed in these patients. Three patients died: one of brain edema due to TTP-specific cerebral microangiopathy and two due to the underlying disease (lupus erythematosus, mixed connective tissue disease). CONCLUSION: Treatment of HUS-TTP is started with fresh-frozen plasma infusions (1-1.5 liters/day), but plasmapheresis should be added 2 days later (3 x 4 liters/week, whereby 2 liters should be given as fresh-frozen plasma). The administration of fresh-frozen plasma must be continued every day. In resistant cases, specific therapy should not be terminated before 4 weeks.