Thrombotic Thrombocytopenic Purpura

Abstract

THROMBOTIC thrombocytopenic purpura (TTP) is an unusual disease manifested clinically by hemolytic anemia, thrombocytopenia, fever, renal involvement and neurologic manifestations, and characterized pathologically by occlusion of small blood vessels in many organs by an homogenous, eosinophilic matter. While the disease is unusual and its etiology is unknown, the condition can no longer be considered rare. Table 1 is a summary of the number of patients with TTP reported since 1925 when the disease was first described.¹The last review²of TTP, published in March 1966, collected 271 cases from the literature. Since then we have found more than 30 new published cases. Neurologic involvement occurs in over 90% of patients with TTP,²and the illness begins as a neurologic one in 47%¹²to 60%³of patients with TTP. Since 1947,⁵almost all reviewers of this interesting disease have reported that the initial neurologic