Thrombotic thrombocytopenic purpura. II. Studies on the hemolytic syndrome in this disease.

Thrombotic thrombocytopenic purpura (generalized platelet thromboses) is an acute febrile disease characterized by a diagnostic triad: thrombocytopenic purpura, hemolytic anemia, and transitory focal neurologic signs. Twenty cases with this condition have been reported so far. Another case is described in which the diagnosis was made ante mortem on the basis of the above criteria. Detailed hematologic, immunologic, and pigmentary studies revealed a hemolytic process unaccompanied by evidence of autoimmunization. The differential diagnosis, etiologic factors, and pathologic physiology of the disease are discussed together with the possibility that a hypersensitivity reaction might be present.

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