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Journal of Blood Disorders Symptoms and Treatments

Aabstract


Review Article

Diagnosis and Management of Thrombotic Thrombocytopenic Purpura

Nahla A. M. Hamed

Correspondence Address :

Nahla A. M. Hamed
Professor of Hematology
Faculty of Medicine
Alexandria University
Egypt
Email: drhamedn@hotmail.com

Received on: July 04, 2018, Accepted on: July 11, 2018, Published on: July 18, 2018

Citation: Nahla A. M. Hamed (2018) Diagnosis and Management of Thrombotic Thrombocytopenic Purpura

Copyright: 2018 Nahla A. M. Hamed, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

  • Abstract

Abstract
TTP is a rare life-threatening disease resulting from absence of functional ADAMTS13. Aggregates of ultralarge vWF multimers-platelet will form microthrombi within the arterial and capillary microvessels of high shear inducing tissue ischemia, platelet consumption and MAHA. Only thrombocytopenia and MAHA without another clinically apparent etiology are required to suspect the diagnosis of TTP. TTP is mainly caused by an autoimmune mechanism. Although the survival rate in acquired TTP exceeds 80%, rapid-onset therapy to prevent further microthrombus formation by targeting the binding of platelets to ultralarge vWF multimers is a potential approach to the treatment. Immunosuppressive therapy (e.g., glucocorticoids and rituximab) inhibits autoantibody formation. Caplacizumab, an anti- vWF humanized single-variable-domain immunoglobulin targets the A1 domain of vWF, preventing interaction with the platelet glycoprotein Ib-IX-V receptor.