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Organ Stiffness in the Work-Up of Myelofibrosis and Philadelphia-Negative Chronic Myeloproliferative Neoplasms

To define the role of spleen stiffness (SS) and liver stiffness (LS) in myelofibrosis and other Philadelphia (Ph)-negative myeloproliferative neoplasms (MPNs), we studied, by ultrasonography (US) and elastography (ES), 70 consecutive patients with myelofibrosis (MF) (no.43), essential thrombocythemi...

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Autores principales: Benedetti, Edoardo, Tavarozzi, Rita, Morganti, Riccardo, Bruno, Benedetto, Bramanti, Emilia, Baratè, Claudia, Balducci, Serena, Iovino, Lorenzo, Ricci, Federica, Ricchiuto, Vittorio, Buda, Gabriele, Galimberti, Sara
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7408647/
https://www.ncbi.nlm.nih.gov/pubmed/32650390
http://dx.doi.org/10.3390/jcm9072149
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author Benedetti, Edoardo
Tavarozzi, Rita
Morganti, Riccardo
Bruno, Benedetto
Bramanti, Emilia
Baratè, Claudia
Balducci, Serena
Iovino, Lorenzo
Ricci, Federica
Ricchiuto, Vittorio
Buda, Gabriele
Galimberti, Sara
author_facet Benedetti, Edoardo
Tavarozzi, Rita
Morganti, Riccardo
Bruno, Benedetto
Bramanti, Emilia
Baratè, Claudia
Balducci, Serena
Iovino, Lorenzo
Ricci, Federica
Ricchiuto, Vittorio
Buda, Gabriele
Galimberti, Sara
author_sort Benedetti, Edoardo
collection PubMed
description To define the role of spleen stiffness (SS) and liver stiffness (LS) in myelofibrosis and other Philadelphia (Ph)-negative myeloproliferative neoplasms (MPNs), we studied, by ultrasonography (US) and elastography (ES), 70 consecutive patients with myelofibrosis (MF) (no.43), essential thrombocythemia (ET) (no.10), and polycythemia vera (PV) (no.17). Overall, the median SS was not different between patients with MF and PV (p = 0.9); however, both MF and PV groups had significantly higher SS than the ET group (p = 0.011 and p = 0.035, respectively) and healthy controls (p < 0.0001 and p = 0.002, respectively). In patients with MF, SS values above 40 kPa were significantly associated with worse progression-free survival (PFS) (p = 0.012; HR = 3.2). SS also correlated with the extension of bone marrow fibrosis (BMF) (p < 0.0001). SS was higher in advanced fibrotic stages MF-2, MF-3 (W.H.O. criteria) than in pre-fibrotic/early fibrotic stages (MF-0, MF-1) (p < 0.0001) and PFS was significantly different in the two cohorts, with values of 63% and 85%, respectively (p = 0.038; HR = 2.61). LS significantly differed between the patient cohort with MF and healthy controls (p = 0.001), but not between the patient cohorts with ET and PV and healthy controls (p = 0.999 and p = 0.101, respectively). We can conclude that organ stiffness adds valuable information to the clinical work-up of MPNs and could be employed to define patients at a higher risk of progression.
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spelling pubmed-74086472020-08-13 Organ Stiffness in the Work-Up of Myelofibrosis and Philadelphia-Negative Chronic Myeloproliferative Neoplasms Benedetti, Edoardo Tavarozzi, Rita Morganti, Riccardo Bruno, Benedetto Bramanti, Emilia Baratè, Claudia Balducci, Serena Iovino, Lorenzo Ricci, Federica Ricchiuto, Vittorio Buda, Gabriele Galimberti, Sara J Clin Med Article To define the role of spleen stiffness (SS) and liver stiffness (LS) in myelofibrosis and other Philadelphia (Ph)-negative myeloproliferative neoplasms (MPNs), we studied, by ultrasonography (US) and elastography (ES), 70 consecutive patients with myelofibrosis (MF) (no.43), essential thrombocythemia (ET) (no.10), and polycythemia vera (PV) (no.17). Overall, the median SS was not different between patients with MF and PV (p = 0.9); however, both MF and PV groups had significantly higher SS than the ET group (p = 0.011 and p = 0.035, respectively) and healthy controls (p < 0.0001 and p = 0.002, respectively). In patients with MF, SS values above 40 kPa were significantly associated with worse progression-free survival (PFS) (p = 0.012; HR = 3.2). SS also correlated with the extension of bone marrow fibrosis (BMF) (p < 0.0001). SS was higher in advanced fibrotic stages MF-2, MF-3 (W.H.O. criteria) than in pre-fibrotic/early fibrotic stages (MF-0, MF-1) (p < 0.0001) and PFS was significantly different in the two cohorts, with values of 63% and 85%, respectively (p = 0.038; HR = 2.61). LS significantly differed between the patient cohort with MF and healthy controls (p = 0.001), but not between the patient cohorts with ET and PV and healthy controls (p = 0.999 and p = 0.101, respectively). We can conclude that organ stiffness adds valuable information to the clinical work-up of MPNs and could be employed to define patients at a higher risk of progression. MDPI 2020-07-08 /pmc/articles/PMC7408647/ /pubmed/32650390 http://dx.doi.org/10.3390/jcm9072149 Text en © 2020 by the authors. 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 (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Benedetti, Edoardo
Tavarozzi, Rita
Morganti, Riccardo
Bruno, Benedetto
Bramanti, Emilia
Baratè, Claudia
Balducci, Serena
Iovino, Lorenzo
Ricci, Federica
Ricchiuto, Vittorio
Buda, Gabriele
Galimberti, Sara
Organ Stiffness in the Work-Up of Myelofibrosis and Philadelphia-Negative Chronic Myeloproliferative Neoplasms
title Organ Stiffness in the Work-Up of Myelofibrosis and Philadelphia-Negative Chronic Myeloproliferative Neoplasms
title_full Organ Stiffness in the Work-Up of Myelofibrosis and Philadelphia-Negative Chronic Myeloproliferative Neoplasms
title_fullStr Organ Stiffness in the Work-Up of Myelofibrosis and Philadelphia-Negative Chronic Myeloproliferative Neoplasms
title_full_unstemmed Organ Stiffness in the Work-Up of Myelofibrosis and Philadelphia-Negative Chronic Myeloproliferative Neoplasms
title_short Organ Stiffness in the Work-Up of Myelofibrosis and Philadelphia-Negative Chronic Myeloproliferative Neoplasms
title_sort organ stiffness in the work-up of myelofibrosis and philadelphia-negative chronic myeloproliferative neoplasms
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7408647/
https://www.ncbi.nlm.nih.gov/pubmed/32650390
http://dx.doi.org/10.3390/jcm9072149
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