Thrombotic thrombocytopenic purpura. II. Principles of therapy and guidelines for management

Thrombotic thrombocytopenic purpura is a life-threatening disorder which requires immediate therapy. Unfortunately, there are no widely accepted therapeutic recommendations for this rare disorder. The literature contains large numbers of uncontrolled and often uncomparable studies of a variety of therapies used in differing combinations. This article attempts to rationalize the therapy of thrombotic thrombocytopenic purpura based on our current understanding of its pathophysiology. The rationale of each therapeutic modality, including plasma therapy, corticosteroids, vincristine, antiplatelet agents, and splenectomy, is discussed. This is followed by an overview of the clinical results reported in the literature for these individual treatments. Finally, overall therapeutic guidelines for the therapy of thrombotic thrombocytopenic purpura are presented. It is hoped that these guidelines will clarify the treatment of this disorder until more definitive therapeutic studies are available.