The Clinical Spectrum of Thrombotic Thrombocytopenic Purpura

Abstract

The clinical syndrome of fever, neurologic abnormalities, renal impairment with laboratory findings of thrombocytopenic and microangiopathic hemolytic anemia is seen in thrombotic thrombocytopenic purpura (TTP) and a variety of disorders associated with thrombotic microangiopathy (TMA). With improved understanding of the pathogenesis of the perturbed metabolic pathway of von Willebrand factor in TTP, the classic Moschcowitz syndrome, now more accurately referred to as idiopathic TTP, can be distinguished from other TMAs. The distinguishing features are useful not only in providing an accurate diagnosis but also help to determine the best therapeutic strategy.

Thrombotic Thrombocytopenic Purpura: A Review of the Disease Entity, its Clinical and Laboratory Features, and Management Strategies

“…These include hemolytic uremic syndrome or MAHA associated with other causes including metastatic cancer, organ transplantation, and connective tissue diseases. 3 Furthermore, not all features of the pentad need be present to diagnose TTP. Treatment initiation, particularly plasma exchange, can significantly decrease the mortality of the disorder and is often initiated prior to confirming the diagnosis.…”

“…These include pregnancy, HIV infection, and obesity. 3 In addition, the use of several medications has been associated with TTP. These include antiplatelet agents (ticlopidine, clopidogrel), antineoplastic agents (mitomycin, cyclosporine and quinine), cocaine, as well as other drugs including antibiotics and statins.…”

“…Conversely, fever and renal involvement are identified in 40% of patients. 3 The multitude of organ systems involved leads to varied symptomatology.…”

Púrpura trombocitopênica trombótica: o papel do fator von Willebrand e da ADAMTS13

“…Dessa forma, os gmFvW no plasma promoveriam a agregação intravascular das plaquetas, resultando em trombose microvascular e hemólise mecânica, particularmente em condições de fluxo sanguíneo caracterizado por alto shear stress, como na microcirculação. 1,5,[9][10][11][12] No entanto, foi somente em 1996 que Furlan et al 13 e Tsai e Lian 4 isolaram uma protease e verificaram que essa era capaz de clivar a ligação peptídica entre a tirosina na posição 1605 e a metionina na posição 1606 no domínio central A2 do FvW. Em um estudo de coorte retrospectivo, estes pesquisadores observaram que os pacientes clinicamente diagnosticados com PTT tinham deficiência desta protease.…”

Thrombotic Thrombocytopenic Purpura

“…1 Microvascular thrombi in arterioles and capillaries of the myocardium consist mainly of platelet aggregates, with only small amounts of fibrin. 1 In thrombotic thrombocytopenic purpura, the histological picture of extensive microvascular thrombotic lesions occurs frequently in the myocardium, but clinical cardiac manifestations are few, and objective findings of myocardial injury have thus far been limited to ECG changes. 1 After acute coronary syndrome occurring in a …”