In this article, we will discuss the Pathogenesis of Thrombotic Thrombocytopenic Purpura. So, let’s get started.
The pathogenesis of thrombotic thrombocytopenic purpura (idiopathic or inherited) is related to deficiency of, or antibodies to, a metalloprotease that cleaves vWF and ADAMTS 13 respectively. Non-cleavage of large vWF molecules results in their persistence which leads to platelet aggregation and adhesion. The manifestations are as a result of localized platelet thrombi and fibrin deposition in microvasculature. Arterioles are filled with hyaline material (fibrin and platelets) and similar material may also be seen beneath the endothelium of the involved vessels. Immunofluorescence studies have shown deposition of immunoglobulins and complement in arterioles indicating an immunologic origin. Microaneurysm of arterioles may also be seen.