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A Novel Morphological Parameter Predicting Fibrotic Evolution in Myeloproliferative Neoplasms: New Evidence and Molecular Insights
Philadelphia-negative chronic myeloproliferative neoplasms (MPNs) represent a group of hematological disorders that are traditionally considered as indistinct slow progressing conditions; still, a subset of cases shows a rapid evolution towards myelofibrotic bone marrow failure. Specific abnormaliti...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9322985/ https://www.ncbi.nlm.nih.gov/pubmed/35887218 http://dx.doi.org/10.3390/ijms23147872 |
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author | Fiorentino, Vincenzo Tralongo, Pietro Martini, Maurizio Betti, Silvia Rossi, Elena Pierconti, Francesco De Stefano, Valerio Larocca, Luigi Maria |
author_facet | Fiorentino, Vincenzo Tralongo, Pietro Martini, Maurizio Betti, Silvia Rossi, Elena Pierconti, Francesco De Stefano, Valerio Larocca, Luigi Maria |
author_sort | Fiorentino, Vincenzo |
collection | PubMed |
description | Philadelphia-negative chronic myeloproliferative neoplasms (MPNs) represent a group of hematological disorders that are traditionally considered as indistinct slow progressing conditions; still, a subset of cases shows a rapid evolution towards myelofibrotic bone marrow failure. Specific abnormalities in the megakaryocyte lineage seem to play a central role in this evolution, especially in the bone marrow fibrosis but also in the induction of myeloproliferation. In this review, we analyze the current knowledge of prognostic factors of MPNs related to their evolution to myelofibrotic bone marrow failure. Moreover, we focused the role of the megakaryocytic lineage in the various stages of MPNs, with updated examples of MPNs in vitro and in vivo models and new therapeutic implications. |
format | Online Article Text |
id | pubmed-9322985 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-93229852022-07-27 A Novel Morphological Parameter Predicting Fibrotic Evolution in Myeloproliferative Neoplasms: New Evidence and Molecular Insights Fiorentino, Vincenzo Tralongo, Pietro Martini, Maurizio Betti, Silvia Rossi, Elena Pierconti, Francesco De Stefano, Valerio Larocca, Luigi Maria Int J Mol Sci Review Philadelphia-negative chronic myeloproliferative neoplasms (MPNs) represent a group of hematological disorders that are traditionally considered as indistinct slow progressing conditions; still, a subset of cases shows a rapid evolution towards myelofibrotic bone marrow failure. Specific abnormalities in the megakaryocyte lineage seem to play a central role in this evolution, especially in the bone marrow fibrosis but also in the induction of myeloproliferation. In this review, we analyze the current knowledge of prognostic factors of MPNs related to their evolution to myelofibrotic bone marrow failure. Moreover, we focused the role of the megakaryocytic lineage in the various stages of MPNs, with updated examples of MPNs in vitro and in vivo models and new therapeutic implications. MDPI 2022-07-17 /pmc/articles/PMC9322985/ /pubmed/35887218 http://dx.doi.org/10.3390/ijms23147872 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/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 (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Fiorentino, Vincenzo Tralongo, Pietro Martini, Maurizio Betti, Silvia Rossi, Elena Pierconti, Francesco De Stefano, Valerio Larocca, Luigi Maria A Novel Morphological Parameter Predicting Fibrotic Evolution in Myeloproliferative Neoplasms: New Evidence and Molecular Insights |
title | A Novel Morphological Parameter Predicting Fibrotic Evolution in Myeloproliferative Neoplasms: New Evidence and Molecular Insights |
title_full | A Novel Morphological Parameter Predicting Fibrotic Evolution in Myeloproliferative Neoplasms: New Evidence and Molecular Insights |
title_fullStr | A Novel Morphological Parameter Predicting Fibrotic Evolution in Myeloproliferative Neoplasms: New Evidence and Molecular Insights |
title_full_unstemmed | A Novel Morphological Parameter Predicting Fibrotic Evolution in Myeloproliferative Neoplasms: New Evidence and Molecular Insights |
title_short | A Novel Morphological Parameter Predicting Fibrotic Evolution in Myeloproliferative Neoplasms: New Evidence and Molecular Insights |
title_sort | novel morphological parameter predicting fibrotic evolution in myeloproliferative neoplasms: new evidence and molecular insights |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9322985/ https://www.ncbi.nlm.nih.gov/pubmed/35887218 http://dx.doi.org/10.3390/ijms23147872 |
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