1. "Thrombotic thrombocytopenic purpura" is the name which we propose for a rare but well-defined disorder which manifests itself clinically as an acute febrile illness and which is characterized by (a) petechiae and ecchymoses, thrombocytopenia, prolonged bleeding time and poor clot retraction, (b) by a severe anemia out of proportion to any observed blood loss, (c) by mild acholuric jaundice, hepato-splenomegaly, (d) by bizarre and intermittent mental and neurologic symptoms and signs, and (e) by a transient leukemoid reaction in the peripheral blood.
2. This clinical picture must be correlated with a remarkable histologic pattern, namely the presence of myriads of platelet thrombi in the small arterioles and capillaries of almost all organs of the body.
3. Eleven such cases have been described in the literature. One case of our own is added.
4. The clinical features of this disease are detailed and the differential diagnosis is discussed. It is emphasized that if the physician is familiar with this syndrome a correct clinical diagnosis may become readily possible.
Thrombotic thrombocytopenic purpura is a relatively new and distinct disease entity manifested clinically by purpura, thrombopenia, hemolytic anemia and bizarre transitory neurologic signs and symptoms. It is characterized histologically by widespread occlusion of the arterioles and capillaries with an amorphous or granular acidophilic material, endothelial proliferation within the vessels and the virtual absence of any inflammatory reaction in the walls of the blood vessel and adjacent tissues.
Three cases occurring in children are reported together with a discussion of our present concepts of the disease.
The clinical diagnosis of thrombotic thrombocytopenic purpura is not difficult provided the symptomatologic triad of thrombopenia, hemolytic anemia and neurologic disturbances are borne in mind. In some instances, diagnosis can be made prior to death by the finding of the characteristic histologic picture in paraffin sections of particles aspirated from the bone marrow or in biopsy specimens of the skin.
It is stressed that the possibility of thrombotic thrombocytopenic purpura should be considered whenever an unusually severe or fulminating purpura, associated with thrombopenia, is encountered.
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