The changing course of thrombotic thrombocytopenic purpura and modern therapy.

Thrombotic thrombocytopenic purpura (TTP) is a multisystem disorder of unknown etiology. Pathologically, there appears to be an abnormal interaction between the vascular endothelium and platelets, but the primary event remains uncertain. While historically, TTP was a fatal disease, dramatic improvement in its outcome has occurred over the past two decades with the development of effective therapy. Plasma infusion or exchange remains the cornerstone of the treatment of TTP, along with corticosteroids, platelet inhibitor drugs, vincristine and splenectomy. This review summarizes the clinical findings, what is known of the pathogenesis and the available therapeutic modalities for TTP. In most cases, remissions can be attained and cures are now common. However, approximately half the patients will relapse. The clinical course at relapse is usually milder than the disease at presentation and less aggressive therapy may be needed. However, relapsing TTP still carries a significant mortality and preventive therapies are not always effective. Further progress may have to await an understanding of the fundamental etiology of this disease.