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Prefibrotic myelofibrosis: treatment algorithm 2018
Prefibrotic myelofibrosis (pre-PMF) is a distinct entity among chronic myeloproliferative neoplasm diagnosed according to the revised 2016 WHO classification. The clinical picture is heterogeneous, ranging from isolated thrombocytosis, mimicking essential thrombocythemia (ET), to symptoms of high-ri...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Nature Publishing Group UK
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6221891/ https://www.ncbi.nlm.nih.gov/pubmed/30405096 http://dx.doi.org/10.1038/s41408-018-0142-z |
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author | Finazzi, Guido Vannucchi, Alessandro M. Barbui, Tiziano |
author_facet | Finazzi, Guido Vannucchi, Alessandro M. Barbui, Tiziano |
author_sort | Finazzi, Guido |
collection | PubMed |
description | Prefibrotic myelofibrosis (pre-PMF) is a distinct entity among chronic myeloproliferative neoplasm diagnosed according to the revised 2016 WHO classification. The clinical picture is heterogeneous, ranging from isolated thrombocytosis, mimicking essential thrombocythemia (ET), to symptoms of high-risk PMF. Retrospective studies showed that survival of patients with pre-PMF is worse than that of ET and better than overt PMF. Whilst a specific prognostic score is lacking, the International Prognostic Scoring System is able to predict survival in pre-PMF patients, yet failing to separate intermediate-1 and -2 groups, and can be used in clinical practice. Each patient should be evaluated for, and interventions adapted to, both life-expectancy and the risk of bleeding and thrombosis. In low-risk patients with expected long survival, observation only is recommended; in cumulated intermediate-1 and -2 risk cases, whose median survival is projected at more than 10 years, treatment is based on symptoms; in high risk cases, with median survival lower than 5 years, intensive management is required. A pragmatic approach to address the risk of bleeding and thrombosis includes: no treatment or low-dose aspirin in asymptomatic patients; aspirin or oral anticoagulation if previous arterial or venous thrombosis, and hydroxyurea as first-line cytoreduction in case of thrombocytosis or leukocytosis. |
format | Online Article Text |
id | pubmed-6221891 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | Nature Publishing Group UK |
record_format | MEDLINE/PubMed |
spelling | pubmed-62218912018-11-08 Prefibrotic myelofibrosis: treatment algorithm 2018 Finazzi, Guido Vannucchi, Alessandro M. Barbui, Tiziano Blood Cancer J Current Treatment Algorithm Prefibrotic myelofibrosis (pre-PMF) is a distinct entity among chronic myeloproliferative neoplasm diagnosed according to the revised 2016 WHO classification. The clinical picture is heterogeneous, ranging from isolated thrombocytosis, mimicking essential thrombocythemia (ET), to symptoms of high-risk PMF. Retrospective studies showed that survival of patients with pre-PMF is worse than that of ET and better than overt PMF. Whilst a specific prognostic score is lacking, the International Prognostic Scoring System is able to predict survival in pre-PMF patients, yet failing to separate intermediate-1 and -2 groups, and can be used in clinical practice. Each patient should be evaluated for, and interventions adapted to, both life-expectancy and the risk of bleeding and thrombosis. In low-risk patients with expected long survival, observation only is recommended; in cumulated intermediate-1 and -2 risk cases, whose median survival is projected at more than 10 years, treatment is based on symptoms; in high risk cases, with median survival lower than 5 years, intensive management is required. A pragmatic approach to address the risk of bleeding and thrombosis includes: no treatment or low-dose aspirin in asymptomatic patients; aspirin or oral anticoagulation if previous arterial or venous thrombosis, and hydroxyurea as first-line cytoreduction in case of thrombocytosis or leukocytosis. Nature Publishing Group UK 2018-11-07 /pmc/articles/PMC6221891/ /pubmed/30405096 http://dx.doi.org/10.1038/s41408-018-0142-z Text en © The Author(s) 2018 Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/. |
spellingShingle | Current Treatment Algorithm Finazzi, Guido Vannucchi, Alessandro M. Barbui, Tiziano Prefibrotic myelofibrosis: treatment algorithm 2018 |
title | Prefibrotic myelofibrosis: treatment algorithm 2018 |
title_full | Prefibrotic myelofibrosis: treatment algorithm 2018 |
title_fullStr | Prefibrotic myelofibrosis: treatment algorithm 2018 |
title_full_unstemmed | Prefibrotic myelofibrosis: treatment algorithm 2018 |
title_short | Prefibrotic myelofibrosis: treatment algorithm 2018 |
title_sort | prefibrotic myelofibrosis: treatment algorithm 2018 |
topic | Current Treatment Algorithm |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6221891/ https://www.ncbi.nlm.nih.gov/pubmed/30405096 http://dx.doi.org/10.1038/s41408-018-0142-z |
work_keys_str_mv | AT finazziguido prefibroticmyelofibrosistreatmentalgorithm2018 AT vannucchialessandrom prefibroticmyelofibrosistreatmentalgorithm2018 AT barbuitiziano prefibroticmyelofibrosistreatmentalgorithm2018 |