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Towards a Personalized Definition of Prognosis in Philadelphia-Negative Myeloproliferative Neoplasms

PURPOSE OF REVIEW: Philadelphia-negative myeloproliferative neoplasms (MPNs) include polycythemia vera (PV), essential thrombocythemia (ET), prefibrotic (pre-), and overt-primary myelofibrosis (primary MF, PMF). PV and ET could evolve into secondary MF (SMF), whose early diagnosis relies on monitori...

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Autores principales: Mora, Barbara, Passamonti, Francesco
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer US 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9499895/
https://www.ncbi.nlm.nih.gov/pubmed/36048275
http://dx.doi.org/10.1007/s11899-022-00672-6
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author Mora, Barbara
Passamonti, Francesco
author_facet Mora, Barbara
Passamonti, Francesco
author_sort Mora, Barbara
collection PubMed
description PURPOSE OF REVIEW: Philadelphia-negative myeloproliferative neoplasms (MPNs) include polycythemia vera (PV), essential thrombocythemia (ET), prefibrotic (pre-), and overt-primary myelofibrosis (primary MF, PMF). PV and ET could evolve into secondary MF (SMF), whose early diagnosis relies on monitoring signs of possible progression. All MPNs have a risk of blast phase (BP), that is associated with a very dismal outcome. Overall survival (OS) is different among MPNs, and disease-specific prognostic scores should be applied for a correct clinical management. In this review, an overview of current prognostic scores in MPNs will be provided. RECENT FINDINGS: The biological complexity of MPNs and its role on the trajectory of disease outcome have led to the design of integrated prognostic models that are nowadays of common use in PMF patients. As for PV and ET, splicing gene mutations could have a detrimental role, but with the limit of the not routinary recommended application of extensive molecular analysis in these diseases. SMF is recognized as a distinct entity compared to PMF, and OS estimates should be calculated by the MYSEC-PM (Myelofibrosis SECondary-prognostic model). Both in PMF and SMF, decisions as selection of patients potentially candidates to allogenic stem cell transplant or that could benefit from an early shift from standard treatment are based not only on conventional prognostic scores, but also on multivariable algorithms. SUMMARY: The expanding landscape of risk prediction for OS, evolution to BP, and SMF progression from PV/ET informs personalized approach to the management of patients affected by MPNs.
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spelling pubmed-94998952022-09-24 Towards a Personalized Definition of Prognosis in Philadelphia-Negative Myeloproliferative Neoplasms Mora, Barbara Passamonti, Francesco Curr Hematol Malig Rep Myeloproliferative Neoplasms (P Bose, Section Editor) PURPOSE OF REVIEW: Philadelphia-negative myeloproliferative neoplasms (MPNs) include polycythemia vera (PV), essential thrombocythemia (ET), prefibrotic (pre-), and overt-primary myelofibrosis (primary MF, PMF). PV and ET could evolve into secondary MF (SMF), whose early diagnosis relies on monitoring signs of possible progression. All MPNs have a risk of blast phase (BP), that is associated with a very dismal outcome. Overall survival (OS) is different among MPNs, and disease-specific prognostic scores should be applied for a correct clinical management. In this review, an overview of current prognostic scores in MPNs will be provided. RECENT FINDINGS: The biological complexity of MPNs and its role on the trajectory of disease outcome have led to the design of integrated prognostic models that are nowadays of common use in PMF patients. As for PV and ET, splicing gene mutations could have a detrimental role, but with the limit of the not routinary recommended application of extensive molecular analysis in these diseases. SMF is recognized as a distinct entity compared to PMF, and OS estimates should be calculated by the MYSEC-PM (Myelofibrosis SECondary-prognostic model). Both in PMF and SMF, decisions as selection of patients potentially candidates to allogenic stem cell transplant or that could benefit from an early shift from standard treatment are based not only on conventional prognostic scores, but also on multivariable algorithms. SUMMARY: The expanding landscape of risk prediction for OS, evolution to BP, and SMF progression from PV/ET informs personalized approach to the management of patients affected by MPNs. Springer US 2022-09-01 2022 /pmc/articles/PMC9499895/ /pubmed/36048275 http://dx.doi.org/10.1007/s11899-022-00672-6 Text en © The Author(s) 2022 https://creativecommons.org/licenses/by/4.0/Open AccessThis 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/ (https://creativecommons.org/licenses/by/4.0/) .
spellingShingle Myeloproliferative Neoplasms (P Bose, Section Editor)
Mora, Barbara
Passamonti, Francesco
Towards a Personalized Definition of Prognosis in Philadelphia-Negative Myeloproliferative Neoplasms
title Towards a Personalized Definition of Prognosis in Philadelphia-Negative Myeloproliferative Neoplasms
title_full Towards a Personalized Definition of Prognosis in Philadelphia-Negative Myeloproliferative Neoplasms
title_fullStr Towards a Personalized Definition of Prognosis in Philadelphia-Negative Myeloproliferative Neoplasms
title_full_unstemmed Towards a Personalized Definition of Prognosis in Philadelphia-Negative Myeloproliferative Neoplasms
title_short Towards a Personalized Definition of Prognosis in Philadelphia-Negative Myeloproliferative Neoplasms
title_sort towards a personalized definition of prognosis in philadelphia-negative myeloproliferative neoplasms
topic Myeloproliferative Neoplasms (P Bose, Section Editor)
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9499895/
https://www.ncbi.nlm.nih.gov/pubmed/36048275
http://dx.doi.org/10.1007/s11899-022-00672-6
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