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Pathogenesis and Therapeutic Mechanisms in Immune Thrombocytopenia (ITP)

Immune thrombocytopenia (ITP) is a complex autoimmune disease characterized by low platelet counts. The pathogenesis of ITP remains unclear although both antibody-mediated and/or T cell-mediated platelet destruction are key processes. In addition, impairment of T cells, cytokine imbalances, and the...

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Autores principales: Zufferey, Anne, Kapur, Rick, Semple, John W.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5332920/
https://www.ncbi.nlm.nih.gov/pubmed/28208757
http://dx.doi.org/10.3390/jcm6020016
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author Zufferey, Anne
Kapur, Rick
Semple, John W.
author_facet Zufferey, Anne
Kapur, Rick
Semple, John W.
author_sort Zufferey, Anne
collection PubMed
description Immune thrombocytopenia (ITP) is a complex autoimmune disease characterized by low platelet counts. The pathogenesis of ITP remains unclear although both antibody-mediated and/or T cell-mediated platelet destruction are key processes. In addition, impairment of T cells, cytokine imbalances, and the contribution of the bone marrow niche have now been recognized to be important. Treatment strategies are aimed at the restoration of platelet counts compatible with adequate hemostasis rather than achieving physiological platelet counts. The first line treatments focus on the inhibition of autoantibody production and platelet degradation, whereas second-line treatments include immunosuppressive drugs, such as Rituximab, and splenectomy. Finally, third-line treatments aim to stimulate platelet production by megakaryocytes. This review discusses the pathophysiology of ITP and how the different treatment modalities affect the pathogenic mechanisms.
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spelling pubmed-53329202017-03-13 Pathogenesis and Therapeutic Mechanisms in Immune Thrombocytopenia (ITP) Zufferey, Anne Kapur, Rick Semple, John W. J Clin Med Review Immune thrombocytopenia (ITP) is a complex autoimmune disease characterized by low platelet counts. The pathogenesis of ITP remains unclear although both antibody-mediated and/or T cell-mediated platelet destruction are key processes. In addition, impairment of T cells, cytokine imbalances, and the contribution of the bone marrow niche have now been recognized to be important. Treatment strategies are aimed at the restoration of platelet counts compatible with adequate hemostasis rather than achieving physiological platelet counts. The first line treatments focus on the inhibition of autoantibody production and platelet degradation, whereas second-line treatments include immunosuppressive drugs, such as Rituximab, and splenectomy. Finally, third-line treatments aim to stimulate platelet production by megakaryocytes. This review discusses the pathophysiology of ITP and how the different treatment modalities affect the pathogenic mechanisms. MDPI 2017-02-09 /pmc/articles/PMC5332920/ /pubmed/28208757 http://dx.doi.org/10.3390/jcm6020016 Text en © 2017 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Zufferey, Anne
Kapur, Rick
Semple, John W.
Pathogenesis and Therapeutic Mechanisms in Immune Thrombocytopenia (ITP)
title Pathogenesis and Therapeutic Mechanisms in Immune Thrombocytopenia (ITP)
title_full Pathogenesis and Therapeutic Mechanisms in Immune Thrombocytopenia (ITP)
title_fullStr Pathogenesis and Therapeutic Mechanisms in Immune Thrombocytopenia (ITP)
title_full_unstemmed Pathogenesis and Therapeutic Mechanisms in Immune Thrombocytopenia (ITP)
title_short Pathogenesis and Therapeutic Mechanisms in Immune Thrombocytopenia (ITP)
title_sort pathogenesis and therapeutic mechanisms in immune thrombocytopenia (itp)
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5332920/
https://www.ncbi.nlm.nih.gov/pubmed/28208757
http://dx.doi.org/10.3390/jcm6020016
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