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

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...

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Main Authors: Daniele Cattaneo, Claudia Vener, Elena Maria Elli, Cristina Bucelli, Nicole Galli, Fabrizio Cavalca, Giuseppe Auteri, Donatella Vincelli, Bruno Martino, Umberto Gianelli, Francesca Palandri, Alessandra Iurlo
Format: Article
Language:English
Published: MDPI AG 2022-04-01
Series:Cancers
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Online Access:https://www.mdpi.com/2072-6694/14/7/1799
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author Daniele Cattaneo
Claudia Vener
Elena Maria Elli
Cristina Bucelli
Nicole Galli
Fabrizio Cavalca
Giuseppe Auteri
Donatella Vincelli
Bruno Martino
Umberto Gianelli
Francesca Palandri
Alessandra Iurlo
author_facet Daniele Cattaneo
Claudia Vener
Elena Maria Elli
Cristina Bucelli
Nicole Galli
Fabrizio Cavalca
Giuseppe Auteri
Donatella Vincelli
Bruno Martino
Umberto Gianelli
Francesca Palandri
Alessandra Iurlo
author_sort Daniele Cattaneo
collection DOAJ
description 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 doaj.art-1b104232ce934241804a0467f2a1a6162023-11-30T23:02:12ZengMDPI AGCancers2072-66942022-04-01147179910.3390/cancers14071799Diabetes and Second Neoplasia Impact on Prognosis in Pre-Fibrotic Primary MyelofibrosisDaniele Cattaneo0Claudia Vener1Elena Maria Elli2Cristina Bucelli3Nicole Galli4Fabrizio Cavalca5Giuseppe Auteri6Donatella Vincelli7Bruno Martino8Umberto Gianelli9Francesca Palandri10Alessandra Iurlo11Hematology Division, Foundation IRCCS Ca’ Granda Ospedale Maggiore Policlinico, 20122 Milan, ItalyDepartment of Oncology and Hemato-Oncology, University of Milan, 20122 Milan, ItalyHematology Division, San Gerardo Hospital, ASST Monza, 20900 Monza, ItalyHematology Division, Foundation IRCCS Ca’ Granda Ospedale Maggiore Policlinico, 20122 Milan, ItalyHematology Division, Foundation IRCCS Ca’ Granda Ospedale Maggiore Policlinico, 20122 Milan, ItalyHematology Division, San Gerardo Hospital, ASST Monza, 20900 Monza, ItalyInstitute of Hematology “L. and A. Seràgnoli”, S. Orsola-Malpighi Hospital, 40138 Bologna, ItalyDivision of Hematology, Azienda Ospedaliera “Bianchi Melacrino Morelli”, 89133 Reggio Calabria, ItalyDivision of Hematology, Azienda Ospedaliera “Bianchi Melacrino Morelli”, 89133 Reggio Calabria, ItalyDepartment of Pathophysiology and Transplantation, University of Milan, 20122 Milan, ItalyInstitute of Hematology “L. and A. Seràgnoli”, S. Orsola-Malpighi Hospital, 40138 Bologna, ItalyHematology Division, Foundation IRCCS Ca’ Granda Ospedale Maggiore Policlinico, 20122 Milan, ItalyThe 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.https://www.mdpi.com/2072-6694/14/7/1799primary myelofibrosispre-fibroticprognosisscoring systemIPSSDIPSS
spellingShingle Daniele Cattaneo
Claudia Vener
Elena Maria Elli
Cristina Bucelli
Nicole Galli
Fabrizio Cavalca
Giuseppe Auteri
Donatella Vincelli
Bruno Martino
Umberto Gianelli
Francesca Palandri
Alessandra Iurlo
Diabetes and Second Neoplasia Impact on Prognosis in Pre-Fibrotic Primary Myelofibrosis
Cancers
primary myelofibrosis
pre-fibrotic
prognosis
scoring system
IPSS
DIPSS
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 primary myelofibrosis
pre-fibrotic
prognosis
scoring system
IPSS
DIPSS
url https://www.mdpi.com/2072-6694/14/7/1799
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