Platelet destruction in immune thrombocytopenia. Understanding the mechanisms

Hamostaseologie. 2016 Aug 3;36(3):187-94. doi: 10.5482/HAMO-14-09-0043. Epub 2015 May 18.

Abstract

Immune thrombocytopenia (ITP) is an autoimmune bleeding disorder characterized by isolated thrombocytopenia. A dysfunctional proliferation of autoreactive T cells is suggested to be responsible for the loss of tolerance to self-platelet antigens in ITP patients. Autoreactive T cells induce uncontrolled proliferation of autoantibody producing B cells leading to persistent anti-platelet autoimmunity in some ITP patients. The autoimmune response causes an increased destruction of platelets by antibody-mediated phagocytosis, complement activation but also by T cell mediated cytotoxicity. In addition, abnormalities in thrombopoiesis and insufficient platelet production due to antibody or T cell mediated megakaryocyte inhibition and destruction contribute to the pathophysiology of ITP. These various effector cell responses may account for the heterogeneity in the clinical manifestation of ITP and also, to success or failure of different treatment strategies. A better understanding of the mechanisms behind ITP will hopefully allow for better diagnostic and, particularly, therapeutic strategies in the future.

Keywords: Platelet; antibody; thrombocytopenia.

Publication types

  • Review

MeSH terms

  • Blood Platelets / immunology*
  • Blood Platelets / pathology
  • Cytokines / immunology
  • Evidence-Based Medicine
  • Humans
  • Models, Immunological*
  • Platelet Activation / immunology*
  • Purpura, Thrombocytopenic, Idiopathic / blood*
  • Purpura, Thrombocytopenic, Idiopathic / immunology*
  • Purpura, Thrombocytopenic, Idiopathic / pathology
  • T-Lymphocytes / immunology*

Substances

  • Cytokines