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Prediction of Survival and Prognosis Migration from Gold-Standard Scores in Myelofibrosis Patients Treated with Ruxolitinib Applying the RR6 Prognostic Model in a Monocentric Real-Life Setting

The wide use of ruxolitinib, approved for treating primary and secondary myelofibrosis (MF), has revolutionized the landscape of these diseases. This molecule can reduce spleen volume and constitutional symptoms, guaranteeing patients a better quality of life and survival or even a valid bridge to b...

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Autores principales: Duminuco, Andrea, Nardo, Antonella, Garibaldi, Bruno, Vetro, Calogero, Longo, Anna, Giallongo, Cesarina, Di Raimondo, Francesco, Palumbo, Giuseppe A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9783796/
https://www.ncbi.nlm.nih.gov/pubmed/36556033
http://dx.doi.org/10.3390/jcm11247418
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author Duminuco, Andrea
Nardo, Antonella
Garibaldi, Bruno
Vetro, Calogero
Longo, Anna
Giallongo, Cesarina
Di Raimondo, Francesco
Palumbo, Giuseppe A.
author_facet Duminuco, Andrea
Nardo, Antonella
Garibaldi, Bruno
Vetro, Calogero
Longo, Anna
Giallongo, Cesarina
Di Raimondo, Francesco
Palumbo, Giuseppe A.
author_sort Duminuco, Andrea
collection PubMed
description The wide use of ruxolitinib, approved for treating primary and secondary myelofibrosis (MF), has revolutionized the landscape of these diseases. This molecule can reduce spleen volume and constitutional symptoms, guaranteeing patients a better quality of life and survival or even a valid bridge to bone marrow transplantation. Despite a rapid response within the first 3 to 6 months of treatment, some patients fail to achieve a significant benefit or lose early response. After ruxolitinib failure, new drugs are available to provide an additional therapeutic option for these patients. However, the correct timing point for deciding on a therapy shift is still an open challenge. Recently, a clinical prognostic score named RR6 (Response to Ruxolitinib after 6 months) was proposed to determine survival after 6 months of treatment with ruxolitinib in patients affected by MF. We applied this model to a cohort of consecutive patients treated at our center to validate the results obtained in terms of median overall survival (mOS): for the low-risk class, mOS was not reached (as in the training cohort); for the intermediate-risk, mOS was 52 months (95% CI 39–106); for the high-risk, it was 33 (95% 8.5–59). Moreover, in addition to the other studies present in the literature, we evaluated how the new RR6 score could better identify primary MF patients at high risk, with a slight or no agreement compared to DIPSS, contrary to what occurs in secondary MF. Thus, we were able to confirm the predictive power of the RR6 model in our series, which might be of help in guiding future therapeutic choices.
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spelling pubmed-97837962022-12-24 Prediction of Survival and Prognosis Migration from Gold-Standard Scores in Myelofibrosis Patients Treated with Ruxolitinib Applying the RR6 Prognostic Model in a Monocentric Real-Life Setting Duminuco, Andrea Nardo, Antonella Garibaldi, Bruno Vetro, Calogero Longo, Anna Giallongo, Cesarina Di Raimondo, Francesco Palumbo, Giuseppe A. J Clin Med Article The wide use of ruxolitinib, approved for treating primary and secondary myelofibrosis (MF), has revolutionized the landscape of these diseases. This molecule can reduce spleen volume and constitutional symptoms, guaranteeing patients a better quality of life and survival or even a valid bridge to bone marrow transplantation. Despite a rapid response within the first 3 to 6 months of treatment, some patients fail to achieve a significant benefit or lose early response. After ruxolitinib failure, new drugs are available to provide an additional therapeutic option for these patients. However, the correct timing point for deciding on a therapy shift is still an open challenge. Recently, a clinical prognostic score named RR6 (Response to Ruxolitinib after 6 months) was proposed to determine survival after 6 months of treatment with ruxolitinib in patients affected by MF. We applied this model to a cohort of consecutive patients treated at our center to validate the results obtained in terms of median overall survival (mOS): for the low-risk class, mOS was not reached (as in the training cohort); for the intermediate-risk, mOS was 52 months (95% CI 39–106); for the high-risk, it was 33 (95% 8.5–59). Moreover, in addition to the other studies present in the literature, we evaluated how the new RR6 score could better identify primary MF patients at high risk, with a slight or no agreement compared to DIPSS, contrary to what occurs in secondary MF. Thus, we were able to confirm the predictive power of the RR6 model in our series, which might be of help in guiding future therapeutic choices. MDPI 2022-12-14 /pmc/articles/PMC9783796/ /pubmed/36556033 http://dx.doi.org/10.3390/jcm11247418 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
Duminuco, Andrea
Nardo, Antonella
Garibaldi, Bruno
Vetro, Calogero
Longo, Anna
Giallongo, Cesarina
Di Raimondo, Francesco
Palumbo, Giuseppe A.
Prediction of Survival and Prognosis Migration from Gold-Standard Scores in Myelofibrosis Patients Treated with Ruxolitinib Applying the RR6 Prognostic Model in a Monocentric Real-Life Setting
title Prediction of Survival and Prognosis Migration from Gold-Standard Scores in Myelofibrosis Patients Treated with Ruxolitinib Applying the RR6 Prognostic Model in a Monocentric Real-Life Setting
title_full Prediction of Survival and Prognosis Migration from Gold-Standard Scores in Myelofibrosis Patients Treated with Ruxolitinib Applying the RR6 Prognostic Model in a Monocentric Real-Life Setting
title_fullStr Prediction of Survival and Prognosis Migration from Gold-Standard Scores in Myelofibrosis Patients Treated with Ruxolitinib Applying the RR6 Prognostic Model in a Monocentric Real-Life Setting
title_full_unstemmed Prediction of Survival and Prognosis Migration from Gold-Standard Scores in Myelofibrosis Patients Treated with Ruxolitinib Applying the RR6 Prognostic Model in a Monocentric Real-Life Setting
title_short Prediction of Survival and Prognosis Migration from Gold-Standard Scores in Myelofibrosis Patients Treated with Ruxolitinib Applying the RR6 Prognostic Model in a Monocentric Real-Life Setting
title_sort prediction of survival and prognosis migration from gold-standard scores in myelofibrosis patients treated with ruxolitinib applying the rr6 prognostic model in a monocentric real-life setting
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9783796/
https://www.ncbi.nlm.nih.gov/pubmed/36556033
http://dx.doi.org/10.3390/jcm11247418
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