Treatment of Thrombotic Thrombocytopenic Purpura

Abstract

This report examines the results of treatment in 21 patients with thrombotic thrombocytopenic purpura (TTP) diagnosed over a 4-year time period (1976-1980) with a review of the possible role of each form of therapy in relationship to the various proposed pathogenic mechanisms. There was a 76.2% (16/21) overall survival with no significant difference in initial hematologic values in patients not surviving. Patients not surviving did not achieve a sustained platelet count greater than 150,000/microliters at any time during their clinical course. 16 patients received steroids, antiplatelet agents and plasma exchange, with a total volume exchanged ranging from 20.8 to 1,455 ml/kg, as part of their treatment protocol. In this group of patients there was an 81.2% (13/16) survival, with 4 patients receiving additional therapy including splenectomy and/or vincristine. There was no correlation between the intensity of plasma exchange and the time to hematologic recovery. It is apparent that controlled clinical trials are necessary to better define the effectiveness of the present forms of therapy.

MECHANISMS IN ENDOCRINOLOGY: The spectrum of haemostatic abnormalities in glucocorticoid excess and defect

“…Most physicians routinely use systematic corticosteroids, such as oral prednisone, for the treatment of thrombotic thrombocytopenic purpura (TTP), and around 60-80% of patients respond with an elevation in platelet count. Several mechanisms have been described for the action of corticosteroids in TTP, including a reduction in autoantibody production, a stabilisation of platelets and endothelial cell membranes and a suppression of reticulo-endothelial phagocytic activity (57,58,59,60,61). However, this is clearly quite a different situation to that wherein patients with normal platelet numbers and function are subject to GT.…”

Indications for therapeutic plasma exchange at a university hospital and a regional blood center

“…Thrombotic thrombocytopenic purpura (TTP), one of the most common syndromes for which TPE is currently utilized, was first observed to respond favorably to TPE (in combination with other therapies) as early as 1959 [10]. The specific effects of TPE on TTP were not widely studied, however, until the 1970s and later [11][12][13][14][15][16]. Numerous publications have documented its efficacy, and TPE is now considered first-line therapy for the treatment of TTP.…”