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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MDPI AG
2022-12-01
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author | Andrea Duminuco Antonella Nardo Bruno Garibaldi Calogero Vetro Anna Longo Cesarina Giallongo Francesco Di Raimondo Giuseppe A. Palumbo |
author_facet | Andrea Duminuco Antonella Nardo Bruno Garibaldi Calogero Vetro Anna Longo Cesarina Giallongo Francesco Di Raimondo Giuseppe A. Palumbo |
author_sort | Andrea Duminuco |
collection | DOAJ |
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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language | English |
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spelling | doaj.art-e0f3f606f3084434bf54918320a36c842023-11-24T15:45:15ZengMDPI AGJournal of Clinical Medicine2077-03832022-12-011124741810.3390/jcm11247418Prediction 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 SettingAndrea Duminuco0Antonella Nardo1Bruno Garibaldi2Calogero Vetro3Anna Longo4Cesarina Giallongo5Francesco Di Raimondo6Giuseppe A. Palumbo7Postgraduate School of Hematology, University of Catania, 95123 Catania, ItalyPostgraduate School of Hematology, University of Catania, 95123 Catania, ItalyPostgraduate School of Hematology, University of Catania, 95123 Catania, ItalyHematology Unit with BMT, A.O.U. Policlinico “G. Rodolico-San Marco”, Via S. Sofia 78, 95123 Catania, ItalyHematology Unit with BMT, A.O.U. Policlinico “G. Rodolico-San Marco”, Via S. Sofia 78, 95123 Catania, ItalyDipartimento di Scienze Mediche, Chirurgiche e Tecnologie Avanzate “G.F. Ingrassia”, University of Catania, 95123 Catania, ItalyHematology Unit with BMT, A.O.U. Policlinico “G. Rodolico-San Marco”, Via S. Sofia 78, 95123 Catania, ItalyDipartimento di Scienze Mediche, Chirurgiche e Tecnologie Avanzate “G.F. Ingrassia”, University of Catania, 95123 Catania, ItalyThe 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.https://www.mdpi.com/2077-0383/11/24/7418RR6 prognostic scoremyelofibrosismodel validationruxolitiniboverall survival |
spellingShingle | Andrea Duminuco Antonella Nardo Bruno Garibaldi Calogero Vetro Anna Longo Cesarina Giallongo Francesco Di Raimondo Giuseppe A. Palumbo 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 Journal of Clinical Medicine RR6 prognostic score myelofibrosis model validation ruxolitinib overall survival |
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 | RR6 prognostic score myelofibrosis model validation ruxolitinib overall survival |
url | https://www.mdpi.com/2077-0383/11/24/7418 |
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