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Low-Risk Myelodysplastic Syndrome Revisited: Morphological, Autoimmune, and Molecular Features as Predictors of Outcome in a Single Center Experience
Low-risk myelodysplastic syndromes (LR-MDS) are a very heterogeneous disease, with extremely variable clinical features and outcome. Therapeutic strategies are still limited and mainly consist of erythropoiesis-stimulating agents (ESAs) and transfusion support. The contribution of molecular lesions...
Autores principales: | , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Frontiers Media S.A.
2022
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8980524/ https://www.ncbi.nlm.nih.gov/pubmed/35392224 http://dx.doi.org/10.3389/fonc.2022.795955 |
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author | Fattizzo, Bruno Levati, Giorgia Virginia Giannotta, Juri Alessandro Cassanello, Giulio Cro, Lilla Marcella Zaninoni, Anna Barbieri, Marzia Croci, Giorgio Alberto Revelli, Nicoletta Barcellini, Wilma |
author_facet | Fattizzo, Bruno Levati, Giorgia Virginia Giannotta, Juri Alessandro Cassanello, Giulio Cro, Lilla Marcella Zaninoni, Anna Barbieri, Marzia Croci, Giorgio Alberto Revelli, Nicoletta Barcellini, Wilma |
author_sort | Fattizzo, Bruno |
collection | PubMed |
description | Low-risk myelodysplastic syndromes (LR-MDS) are a very heterogeneous disease, with extremely variable clinical features and outcome. Therapeutic strategies are still limited and mainly consist of erythropoiesis-stimulating agents (ESAs) and transfusion support. The contribution of molecular lesions and of autoimmune phenomena to pathogenesis and clinical course, including leukemic evolution, is a field of open investigation. We analyzed data from a cohort of 226 patients with LR-MDS followed at our center in the last 20 years, focusing on morphological, immunological (antiplatelets and anti-erythrocyte autoantibodies, anti-erythroblast antibodies), and molecular features. Hypoplastic bone marrow was found in 7% of the cases correlating with younger age, deeper cytopenia, lower dysplasia, and worse response to ESAs. A marker of autoimmunity was observed in 46% of the tested cases, who were younger, were less frequent dysplastic changes, and responded better to ESAs and steroids. Finally, 68% of the tested cases displayed at least one somatic mutation, most commonly SF3B1, TET2, ASXL1, and SRSF2, associated with older age, presence of neutropenia, and lower response to ESAs. Leukemic evolution (2.2%) was associated with presence of somatic mutations, and survival was favorably related to response to ESAs and transfusion independence. Overall, granular evaluation and re-evaluation are pivotal in LR-MDS patients to optimize clinical management. |
format | Online Article Text |
id | pubmed-8980524 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-89805242022-04-06 Low-Risk Myelodysplastic Syndrome Revisited: Morphological, Autoimmune, and Molecular Features as Predictors of Outcome in a Single Center Experience Fattizzo, Bruno Levati, Giorgia Virginia Giannotta, Juri Alessandro Cassanello, Giulio Cro, Lilla Marcella Zaninoni, Anna Barbieri, Marzia Croci, Giorgio Alberto Revelli, Nicoletta Barcellini, Wilma Front Oncol Oncology Low-risk myelodysplastic syndromes (LR-MDS) are a very heterogeneous disease, with extremely variable clinical features and outcome. Therapeutic strategies are still limited and mainly consist of erythropoiesis-stimulating agents (ESAs) and transfusion support. The contribution of molecular lesions and of autoimmune phenomena to pathogenesis and clinical course, including leukemic evolution, is a field of open investigation. We analyzed data from a cohort of 226 patients with LR-MDS followed at our center in the last 20 years, focusing on morphological, immunological (antiplatelets and anti-erythrocyte autoantibodies, anti-erythroblast antibodies), and molecular features. Hypoplastic bone marrow was found in 7% of the cases correlating with younger age, deeper cytopenia, lower dysplasia, and worse response to ESAs. A marker of autoimmunity was observed in 46% of the tested cases, who were younger, were less frequent dysplastic changes, and responded better to ESAs and steroids. Finally, 68% of the tested cases displayed at least one somatic mutation, most commonly SF3B1, TET2, ASXL1, and SRSF2, associated with older age, presence of neutropenia, and lower response to ESAs. Leukemic evolution (2.2%) was associated with presence of somatic mutations, and survival was favorably related to response to ESAs and transfusion independence. Overall, granular evaluation and re-evaluation are pivotal in LR-MDS patients to optimize clinical management. Frontiers Media S.A. 2022-03-22 /pmc/articles/PMC8980524/ /pubmed/35392224 http://dx.doi.org/10.3389/fonc.2022.795955 Text en Copyright © 2022 Fattizzo, Levati, Giannotta, Cassanello, Cro, Zaninoni, Barbieri, Croci, Revelli and Barcellini https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Oncology Fattizzo, Bruno Levati, Giorgia Virginia Giannotta, Juri Alessandro Cassanello, Giulio Cro, Lilla Marcella Zaninoni, Anna Barbieri, Marzia Croci, Giorgio Alberto Revelli, Nicoletta Barcellini, Wilma Low-Risk Myelodysplastic Syndrome Revisited: Morphological, Autoimmune, and Molecular Features as Predictors of Outcome in a Single Center Experience |
title | Low-Risk Myelodysplastic Syndrome Revisited: Morphological, Autoimmune, and Molecular Features as Predictors of Outcome in a Single Center Experience |
title_full | Low-Risk Myelodysplastic Syndrome Revisited: Morphological, Autoimmune, and Molecular Features as Predictors of Outcome in a Single Center Experience |
title_fullStr | Low-Risk Myelodysplastic Syndrome Revisited: Morphological, Autoimmune, and Molecular Features as Predictors of Outcome in a Single Center Experience |
title_full_unstemmed | Low-Risk Myelodysplastic Syndrome Revisited: Morphological, Autoimmune, and Molecular Features as Predictors of Outcome in a Single Center Experience |
title_short | Low-Risk Myelodysplastic Syndrome Revisited: Morphological, Autoimmune, and Molecular Features as Predictors of Outcome in a Single Center Experience |
title_sort | low-risk myelodysplastic syndrome revisited: morphological, autoimmune, and molecular features as predictors of outcome in a single center experience |
topic | Oncology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8980524/ https://www.ncbi.nlm.nih.gov/pubmed/35392224 http://dx.doi.org/10.3389/fonc.2022.795955 |
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