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Diabetes and Second Neoplasia Impact on Prognosis in Pre-Fibrotic Primary Myelofibrosis

SIMPLE SUMMARY: The 2016 WHO-revised classification of MPNs recognized pre-fibrotic PMF (pre-PMF) as a distinct clinical entity from both overt fibrotic PMF (overt PMF) and essential thrombocythemia (ET). In fact, while the initial presentation of pre-PMF is often an isolated thrombocytosis, thus mi...

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Autores principales: Cattaneo, Daniele, Vener, Claudia, Elli, Elena Maria, Bucelli, Cristina, Galli, Nicole, Cavalca, Fabrizio, Auteri, Giuseppe, Vincelli, Donatella, Martino, Bruno, Gianelli, Umberto, Palandri, Francesca, Iurlo, Alessandra
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
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Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8997979/
https://www.ncbi.nlm.nih.gov/pubmed/35406571
http://dx.doi.org/10.3390/cancers14071799
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author Cattaneo, Daniele
Vener, Claudia
Elli, Elena Maria
Bucelli, Cristina
Galli, Nicole
Cavalca, Fabrizio
Auteri, Giuseppe
Vincelli, Donatella
Martino, Bruno
Gianelli, Umberto
Palandri, Francesca
Iurlo, Alessandra
author_facet Cattaneo, Daniele
Vener, Claudia
Elli, Elena Maria
Bucelli, Cristina
Galli, Nicole
Cavalca, Fabrizio
Auteri, Giuseppe
Vincelli, Donatella
Martino, Bruno
Gianelli, Umberto
Palandri, Francesca
Iurlo, Alessandra
author_sort Cattaneo, Daniele
collection PubMed
description SIMPLE SUMMARY: The 2016 WHO-revised classification of MPNs recognized pre-fibrotic PMF (pre-PMF) as a distinct clinical entity from both overt fibrotic PMF (overt PMF) and essential thrombocythemia (ET). In fact, while the initial presentation of pre-PMF is often an isolated thrombocytosis, thus mimicking ET, its course may be symptomatic in a non-negligible number of cases. Conversely, overt PMF patients are enriched in higher-risk categories, thus suggesting a greater propensity for disease progression than pre-PMF. Importantly, median survival is significantly reduced in overt PMF vs. pre-PMF, thereby reinforcing the appropriateness of making this distinction in clinical practice. Nevertheless, a specific prognostic model for pre-PMF is still lacking, except for thrombotic risk. The aim of the present study was therefore to identify covariates other than those commonly related to PMF, which can better define prognosis in pre-PMF patients in the real-world setting, thus resulting in more personalized and efficient therapeutic approaches. ABSTRACT: The 2016 WHO classification recognized pre-fibrotic primary myelofibrosis (pre-PMF) as a distinct entity. Nevertheless, a prognostic model specific for pre-PMF is still lacking. Our aim was to identify the most relevant clinical, histological, and driver mutation information at diagnosis to evaluate outcomes in pre-PMF patients in the real-world setting. We firstly assessed the association between IPSS or DIPSS at diagnosis and response variables in 378 pre-PMF patients. A strict association was observed between IPSS and DIPSS and occurrence of death. Other analyzed endpoints were not associated with IPSS or DIPSS as thrombo-hemorrhagic events at diagnosis or during follow-up, or did not show a clinical plausibility, as transformation into acute leukemia or overt PMF. The only covariates which were significantly associated with death were diabetes and second neoplasia, and were therefore included in two different prognostic settings: the first based on IPSS at diagnosis [class 1 vs. 0, OR (95%CIs): 3.34 (1.85–6.04); class 2 vs. 0, OR (95%CIs): 12.55 (5.04–31.24)], diabetes [OR (95%CIs): 2.95 (1.41–6.18)], and second neoplasia [OR (95%CIs): 2.88 (1.63–5.07)]; the second with DIPSS at diagnosis [class 1 vs. 0, OR (95%CIs): 3.40 (1.89–6.10); class 2 vs. 0, OR (95%CIs): 25.65 (7.62–86.42)], diabetes [OR (95%CIs): 2.89 (1.37–6.09)], and second neoplasia [OR (95%CIs): 2.97 (1.69–5.24)]. In conclusion, our study underlines the importance of other additional risk factors, such as diabetes and second neoplasia, to be evaluated, together with IPSS and DIPSS, to better define prognosis in pre-PMF patients.
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spelling pubmed-89979792022-04-12 Diabetes and Second Neoplasia Impact on Prognosis in Pre-Fibrotic Primary Myelofibrosis Cattaneo, Daniele Vener, Claudia Elli, Elena Maria Bucelli, Cristina Galli, Nicole Cavalca, Fabrizio Auteri, Giuseppe Vincelli, Donatella Martino, Bruno Gianelli, Umberto Palandri, Francesca Iurlo, Alessandra Cancers (Basel) Article SIMPLE SUMMARY: The 2016 WHO-revised classification of MPNs recognized pre-fibrotic PMF (pre-PMF) as a distinct clinical entity from both overt fibrotic PMF (overt PMF) and essential thrombocythemia (ET). In fact, while the initial presentation of pre-PMF is often an isolated thrombocytosis, thus mimicking ET, its course may be symptomatic in a non-negligible number of cases. Conversely, overt PMF patients are enriched in higher-risk categories, thus suggesting a greater propensity for disease progression than pre-PMF. Importantly, median survival is significantly reduced in overt PMF vs. pre-PMF, thereby reinforcing the appropriateness of making this distinction in clinical practice. Nevertheless, a specific prognostic model for pre-PMF is still lacking, except for thrombotic risk. The aim of the present study was therefore to identify covariates other than those commonly related to PMF, which can better define prognosis in pre-PMF patients in the real-world setting, thus resulting in more personalized and efficient therapeutic approaches. ABSTRACT: The 2016 WHO classification recognized pre-fibrotic primary myelofibrosis (pre-PMF) as a distinct entity. Nevertheless, a prognostic model specific for pre-PMF is still lacking. Our aim was to identify the most relevant clinical, histological, and driver mutation information at diagnosis to evaluate outcomes in pre-PMF patients in the real-world setting. We firstly assessed the association between IPSS or DIPSS at diagnosis and response variables in 378 pre-PMF patients. A strict association was observed between IPSS and DIPSS and occurrence of death. Other analyzed endpoints were not associated with IPSS or DIPSS as thrombo-hemorrhagic events at diagnosis or during follow-up, or did not show a clinical plausibility, as transformation into acute leukemia or overt PMF. The only covariates which were significantly associated with death were diabetes and second neoplasia, and were therefore included in two different prognostic settings: the first based on IPSS at diagnosis [class 1 vs. 0, OR (95%CIs): 3.34 (1.85–6.04); class 2 vs. 0, OR (95%CIs): 12.55 (5.04–31.24)], diabetes [OR (95%CIs): 2.95 (1.41–6.18)], and second neoplasia [OR (95%CIs): 2.88 (1.63–5.07)]; the second with DIPSS at diagnosis [class 1 vs. 0, OR (95%CIs): 3.40 (1.89–6.10); class 2 vs. 0, OR (95%CIs): 25.65 (7.62–86.42)], diabetes [OR (95%CIs): 2.89 (1.37–6.09)], and second neoplasia [OR (95%CIs): 2.97 (1.69–5.24)]. In conclusion, our study underlines the importance of other additional risk factors, such as diabetes and second neoplasia, to be evaluated, together with IPSS and DIPSS, to better define prognosis in pre-PMF patients. MDPI 2022-04-01 /pmc/articles/PMC8997979/ /pubmed/35406571 http://dx.doi.org/10.3390/cancers14071799 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 Article
Cattaneo, Daniele
Vener, Claudia
Elli, Elena Maria
Bucelli, Cristina
Galli, Nicole
Cavalca, Fabrizio
Auteri, Giuseppe
Vincelli, Donatella
Martino, Bruno
Gianelli, Umberto
Palandri, Francesca
Iurlo, Alessandra
Diabetes and Second Neoplasia Impact on Prognosis in Pre-Fibrotic Primary Myelofibrosis
title Diabetes and Second Neoplasia Impact on Prognosis in Pre-Fibrotic Primary Myelofibrosis
title_full Diabetes and Second Neoplasia Impact on Prognosis in Pre-Fibrotic Primary Myelofibrosis
title_fullStr Diabetes and Second Neoplasia Impact on Prognosis in Pre-Fibrotic Primary Myelofibrosis
title_full_unstemmed Diabetes and Second Neoplasia Impact on Prognosis in Pre-Fibrotic Primary Myelofibrosis
title_short Diabetes and Second Neoplasia Impact on Prognosis in Pre-Fibrotic Primary Myelofibrosis
title_sort diabetes and second neoplasia impact on prognosis in pre-fibrotic primary myelofibrosis
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8997979/
https://www.ncbi.nlm.nih.gov/pubmed/35406571
http://dx.doi.org/10.3390/cancers14071799
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