Thrombotic thrombocytopenic purpura in a previously splenectomized patient

Abstract

Idiopathic thrombocytopenic purpura was diagnosed in a 26 year old man who had rectal bleeding and marked thrombocytopenia (10,000 platelets/mm3). Complete recovery followed treatment with steroids and splenectomy. There was no clinical, laboratory or histopathologic evidence of thrombotic thrombocytopenic purpura. Several months later typical thrombotic thrombocytopenic purpura developed; recovery followed treatment with steroids, aspirin and dipyridamole. The presence of Howell-Jolly bodies and a negative scan indicated that an accessory spleen was not present. Since thrombotic thrombocytopenic purpura developed in the absence of a spleen in this case, it may be that in some, if not all, instances of thrombotic thrombocytopenic purpura the spleen is not importantly related to the pathogenesis of thrombotic thrombocytopenic purpura; this is in contrast to the situation in idiopathic thrombocytopenic purpura. Benefits attributed to splenectomy in thrombotic thrombocytopenic purpura, therefore, may in fact be due to the platelet inhibitory properties of common anesthetic agents, or to some other factor in the surgical procedure rather than to removal of the spleen per se.