Thrombotic thrombocytopenic purpura. A review

Thrombotic thrombocytopenic purpura (Moschcowitz's disease) is an uncommon, life-threatening disorder of unknown cause. It consists of fluctuating symptoms including: haemolytic anemia, fever, thrombocytopenia, neurological and mental disturbances, and renal dysfunctions. The high mortality of this disease necessitates rapid diagnosis and immediate therapy. The clinical manifestations and diagnostic criteria are reviewed. A variety of theories have been proposed to explain its clinical and pathological manifestations, but the pathophysiology remains poorly understood. The present knowledge of the pathophysiology is discussed in detail. Failure to define the pathophysiology is a reason why there are no widely accepted therapeutic recommendations. The modes of therapy of the disease in relation to current understanding of its nature are discussed. The effect of exchange transfusions, plasma infusions, plasmapheresis and plasma exchange, corticosteroid therapy, immunosuppressive drugs, antiplatelet therapy and splenectomy on clinical course are reviewed.