Any talk about thrombotic thrombocytopenic purpura (T.T.P.) should begin with the paper by Dr. Moschowitz from New York [l]. In 1925, he described a febrile illness with anemia and hyaline thrombosis of small terminal arterioles and capillaries. Up to that point it was an undescribed disease, and the patient he describes typifies what we see today.
extremity weakness, a fever of 104 F, anemia, multiple petechiae, and somewhat impaired renal function, with red blood cells in her urine. Her clinical course was a rapid one and she expired 13 days following the time of presentation. Up until the 1 9 7 0 ' ~~ T.T.P. was considered a fulminant illness, and if one looks at the reviews by Amorosi and Ultmann in 1966 [2], it was estimated that 90% of these patients died as a result of their disease. fever in the range of 102 , some manifestations of bleeding and thrombocytopenia, platelet counts usually being less than 50,00O/ul, traumatic hemolytic anemia, a reticulocytosis, neurologic manifestations which are characteristically transient , and may take any form including hemiparesis or confusion, and lastly, renal manifestations. The renal manifestations in T.T.P. are characteristically mild. Ordinarily, these patients have minimal elevations of BUN and creatinine; they are not oliguric, but they do have some sediment findings , proteinuria and microscopic hematuria. Pathologic feature in T.T. P. are involvement of the small arterioles and capillaries, showing hyaline microthrombi, with no fibrinoid necrosis. These particular lesions are thought to cause the syndrome in that they occur in the microcirculation, they are responsible for the hemolytic anemia that is seen, and probably the thrombi contain platelets, or platelet aggregates. These lesions are widespread in T.T.P., occurring in all organ systems of the body, central nervous system, kidneys, lungs, and so on.