Thrombotic Thrombocytopenic Purpura

Abstract

A case of thrombotic thrombocytopenic purpura in a 50-year-old woman is described. Almost the whole course of the disease, lasting 18 months, was characterized by a bizarre neurological disorder, and the haematological manifestations first appeared at a late stage.In many organs a vast number of arterioles and capillaries contained plugs of a fibrinoid material, and fibrinoid was subendothelially accumulated in a few of these vessels; but, in addition, mediumsized arteries of the myocardium were also obstructed by this same material. There were also verrucal endocardiosis of the mitral valve and slight thickening of the glomerular basement membranes. The striking diffusion of a pathological substance through damaged cerebral vessel walls into the nervous tissue seems to be a significant contribution to the understanding of the pathogenesis of the vascular pathology of thrombotic thrombocytopenic purpura.

Platelet secretory products may contribute to neuronal injury.

“…Disruption of the blood-brain barrier in the cerebral vessels containing the platelet thrombi has also been observed. 23 Although the rarity of cerebral infarction distal to an occluding thrombus in thrombotic thrombocytopenic purpura may be explained by good collaterization, this does not fully explain the perivascular neuronal changes. There remains the possibility that platelet secretory products derived from luminal thrombi cause the damage to the surrounding neurons.…”