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Acquired Aplastic Anemia as a Clonal Disorder of Hematopoietic Stem Cells
Aplastic anemia is rare disorder presenting with bone marrow failure syndrome due to autoimmune destruction of early hematopoietic stem cells (HSCs) and stem cell progenitors. Recent advances in newer genomic sequencing and other molecular techniques have contributed to a better understanding of the...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer US
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7253510/ https://www.ncbi.nlm.nih.gov/pubmed/32270433 http://dx.doi.org/10.1007/s12015-020-09971-y |
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author | Brzeźniakiewicz-Janus, Katarzyna Rupa-Matysek, Joanna Gil, Lidia |
author_facet | Brzeźniakiewicz-Janus, Katarzyna Rupa-Matysek, Joanna Gil, Lidia |
author_sort | Brzeźniakiewicz-Janus, Katarzyna |
collection | PubMed |
description | Aplastic anemia is rare disorder presenting with bone marrow failure syndrome due to autoimmune destruction of early hematopoietic stem cells (HSCs) and stem cell progenitors. Recent advances in newer genomic sequencing and other molecular techniques have contributed to a better understanding of the pathogenesis of aplastic anemia with respect to the inflammaging, somatic mutations, cytogenetic abnormalities and defective telomerase functions of HSCs. These have been summarized in this review and may be helpful in differentiating aplastic anemia from hypocellular myelodysplastic syndrome. Furthermore, responses to immunosuppressive therapy and outcomes may be determined by molecular pathogenesis of HSCs autoimmune destruction, as well as treatment personalization in the future. |
format | Online Article Text |
id | pubmed-7253510 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Springer US |
record_format | MEDLINE/PubMed |
spelling | pubmed-72535102020-06-05 Acquired Aplastic Anemia as a Clonal Disorder of Hematopoietic Stem Cells Brzeźniakiewicz-Janus, Katarzyna Rupa-Matysek, Joanna Gil, Lidia Stem Cell Rev Rep Article Aplastic anemia is rare disorder presenting with bone marrow failure syndrome due to autoimmune destruction of early hematopoietic stem cells (HSCs) and stem cell progenitors. Recent advances in newer genomic sequencing and other molecular techniques have contributed to a better understanding of the pathogenesis of aplastic anemia with respect to the inflammaging, somatic mutations, cytogenetic abnormalities and defective telomerase functions of HSCs. These have been summarized in this review and may be helpful in differentiating aplastic anemia from hypocellular myelodysplastic syndrome. Furthermore, responses to immunosuppressive therapy and outcomes may be determined by molecular pathogenesis of HSCs autoimmune destruction, as well as treatment personalization in the future. Springer US 2020-04-08 2020 /pmc/articles/PMC7253510/ /pubmed/32270433 http://dx.doi.org/10.1007/s12015-020-09971-y Text en © The Author(s) 2020 Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. |
spellingShingle | Article Brzeźniakiewicz-Janus, Katarzyna Rupa-Matysek, Joanna Gil, Lidia Acquired Aplastic Anemia as a Clonal Disorder of Hematopoietic Stem Cells |
title | Acquired Aplastic Anemia as a Clonal Disorder of Hematopoietic Stem Cells |
title_full | Acquired Aplastic Anemia as a Clonal Disorder of Hematopoietic Stem Cells |
title_fullStr | Acquired Aplastic Anemia as a Clonal Disorder of Hematopoietic Stem Cells |
title_full_unstemmed | Acquired Aplastic Anemia as a Clonal Disorder of Hematopoietic Stem Cells |
title_short | Acquired Aplastic Anemia as a Clonal Disorder of Hematopoietic Stem Cells |
title_sort | acquired aplastic anemia as a clonal disorder of hematopoietic stem cells |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7253510/ https://www.ncbi.nlm.nih.gov/pubmed/32270433 http://dx.doi.org/10.1007/s12015-020-09971-y |
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