Aug. 8, 1991 | WILLIAM R. BELL, M.D., HAYDEN G. BRAINE, M.D., PAUL M. NESS, M.D., AND THOMAS S. KICKLER, M.D.
The study by Bell et al. (1991) reports on the clinical experience of treating 108 patients with thrombotic thrombocytopenic purpura–hemolytic uremic syndrome (TTP-HUS) at Johns Hopkins Hospital from September 1979 to December 1990. TTP-HUS is characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, central nervous system abnormalities, and renal dysfunction, with a mortality rate that approached 100% in early reports. The treatment protocol included 200 mg of prednisone daily for patients with minimal symptoms and no central nervous system involvement, and a combination of prednisone and plasma exchange for patients with rapid clinical deterioration or central nervous system symptoms.
The results showed that 91% of the patients survived. Prednisone alone was effective in 30 patients with mild TTP-HUS, while plasma exchange plus prednisone was given to 78 patients with more severe cases, resulting in 67 relapses and 8 deaths. Relapses occurred in 22 of 36 patients receiving maintenance plasma infusions. Splenectomy and treatment with aspirin and dipryidamole were ineffective for those with poor responses to plasma exchange. None of the 71 patients tested had positive cultures for O157:H7 Escherichia coli. Nine percent of the patients were pregnant, and none gave birth to infants with TTP-HUS.
The study concludes that effective treatment with a 91% survival rate is available for patients with TTP-HUS. The findings highlight the importance of early recognition, diagnosis, and timely intervention, as well as the need for further research to understand the underlying causes of this devastating disease.The study by Bell et al. (1991) reports on the clinical experience of treating 108 patients with thrombotic thrombocytopenic purpura–hemolytic uremic syndrome (TTP-HUS) at Johns Hopkins Hospital from September 1979 to December 1990. TTP-HUS is characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, central nervous system abnormalities, and renal dysfunction, with a mortality rate that approached 100% in early reports. The treatment protocol included 200 mg of prednisone daily for patients with minimal symptoms and no central nervous system involvement, and a combination of prednisone and plasma exchange for patients with rapid clinical deterioration or central nervous system symptoms.
The results showed that 91% of the patients survived. Prednisone alone was effective in 30 patients with mild TTP-HUS, while plasma exchange plus prednisone was given to 78 patients with more severe cases, resulting in 67 relapses and 8 deaths. Relapses occurred in 22 of 36 patients receiving maintenance plasma infusions. Splenectomy and treatment with aspirin and dipryidamole were ineffective for those with poor responses to plasma exchange. None of the 71 patients tested had positive cultures for O157:H7 Escherichia coli. Nine percent of the patients were pregnant, and none gave birth to infants with TTP-HUS.
The study concludes that effective treatment with a 91% survival rate is available for patients with TTP-HUS. The findings highlight the importance of early recognition, diagnosis, and timely intervention, as well as the need for further research to understand the underlying causes of this devastating disease.