Thrombotic thrombocytopenic purpura. Treatment with corticosteroids and splenectomy

Abstract

Since Moschcowitz first described thrombotic thrombocytopenic purpura (TTP) in 1925, 1 almost 300 cases have been reported, and complete reviews were published recently. 2,3 In the majority of cases, the disease was characterized by its rapid onset, fulminating course, and fatal outcome; diagnosis was established only at autopsy. Only 20 patients are reported alive and clinically cured, or in remission. 2-18 Eleven of these have received corticosteroid therapy and undergone splenectomy. 3-12 Although all cases of TTP have not been reported, survival is so uncommon that most cases with remission have probably been reviewed. If this is true, the survival percentage is lowered further. The purpose of this paper is to report three additional living patients treated with large doses of corticosteroids and splenectomy. To date, remission of ten, five, and two years' duration have been achieved. The patients have remained free of symptoms and have not been maintained