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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...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2017
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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. |
format | Online Article Text |
id | pubmed-5332920 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
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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