Thrombotic thrombocytopenic purpura.

Thrombotic thrombocytopenic purpura (TTP) is an uncommon disorder that was considered a fatal disease until the early 1960s. This syndrome is complex and controversial. It has been characterized by thrombocytopenic purpura, hemolytic anemia, neurologic symptoms, renal disease, and fever. The causative factor appears to be an increase of a platelet-aggregating factor or a deficiency in some platelet-aggregation inhibitor. The disease process seen is usually caused by endothelial damage mediated by a variety of stimuli. This endothelial damage then results in the formation of microscopic thrombi composed primarily of platelets and found in the small blood vessels of many organs. TTP can occur in any age group but is most often seen in patients 30 to 50 years of age. It is a very serious and potentially fatal acute disorder, but greater than 50% of the patients with this syndrome will have long-lasting remission if proper therapy is initiated and maintained. This article presents clinical findings and symptoms, methods of diagnosis, and appropriate therapeutic interventions for this disorder.