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Treatment and management of myelofibrosis in the era of JAK inhibitors
Myelofibrosis (MF) can present as a primary disorder or evolve from polycythemia vera (PV) or essential thrombocythemia (ET) to post-PV MF or post-ET MF, respectively. MF is characterized by bone marrow fibrosis, splenomegaly, leukoerythroblastosis, extramedullary hematopoiesis, and a collection of...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Dove Medical Press
2013
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3753053/ https://www.ncbi.nlm.nih.gov/pubmed/23990704 http://dx.doi.org/10.2147/BTT.S34942 |
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author | Keohane, Clodagh Radia, Deepti H Harrison, Claire N |
author_facet | Keohane, Clodagh Radia, Deepti H Harrison, Claire N |
author_sort | Keohane, Clodagh |
collection | PubMed |
description | Myelofibrosis (MF) can present as a primary disorder or evolve from polycythemia vera (PV) or essential thrombocythemia (ET) to post-PV MF or post-ET MF, respectively. MF is characterized by bone marrow fibrosis, splenomegaly, leukoerythroblastosis, extramedullary hematopoiesis, and a collection of debilitating symptoms. Until recently, the therapeutic options for patients with MF consisted of allogeneic hematopoietic stem cell transplant (alloHSCT), the use of cytoreductive agents (ie, hydroxyurea), splenectomy and splenic irradiation for treatment of splenomegaly, and management of anemia with transfusions, erythropoiesis-stimulating agents (ESAs), androgens, and immunomodulatory agents. However, with increased understanding of the pathogenesis of MF resulting from dysregulated Janus kinase (JAK) signaling, new targeted JAK inhibitor therapies, such as ruxolitinib, are now available. The purpose of this article is to review the clinical features of MF, discuss the use and future of JAK inhibitors, reassess when and how to use conventional MF treatments in the context of JAK inhibitors, and provide a perspective on the future of MF treatment. |
format | Online Article Text |
id | pubmed-3753053 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2013 |
publisher | Dove Medical Press |
record_format | MEDLINE/PubMed |
spelling | pubmed-37530532013-08-29 Treatment and management of myelofibrosis in the era of JAK inhibitors Keohane, Clodagh Radia, Deepti H Harrison, Claire N Biologics Review Myelofibrosis (MF) can present as a primary disorder or evolve from polycythemia vera (PV) or essential thrombocythemia (ET) to post-PV MF or post-ET MF, respectively. MF is characterized by bone marrow fibrosis, splenomegaly, leukoerythroblastosis, extramedullary hematopoiesis, and a collection of debilitating symptoms. Until recently, the therapeutic options for patients with MF consisted of allogeneic hematopoietic stem cell transplant (alloHSCT), the use of cytoreductive agents (ie, hydroxyurea), splenectomy and splenic irradiation for treatment of splenomegaly, and management of anemia with transfusions, erythropoiesis-stimulating agents (ESAs), androgens, and immunomodulatory agents. However, with increased understanding of the pathogenesis of MF resulting from dysregulated Janus kinase (JAK) signaling, new targeted JAK inhibitor therapies, such as ruxolitinib, are now available. The purpose of this article is to review the clinical features of MF, discuss the use and future of JAK inhibitors, reassess when and how to use conventional MF treatments in the context of JAK inhibitors, and provide a perspective on the future of MF treatment. Dove Medical Press 2013 2013-08-20 /pmc/articles/PMC3753053/ /pubmed/23990704 http://dx.doi.org/10.2147/BTT.S34942 Text en © 2013 Keohane et al. This work is published by Dove Medical Press Ltd, and licensed under Creative Commons Attribution – Non Commercial (unported, v3.0) License The full terms of the License are available at http://creativecommons.org/licenses/by-nc/3.0/. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Ltd, provided the work is properly attributed. |
spellingShingle | Review Keohane, Clodagh Radia, Deepti H Harrison, Claire N Treatment and management of myelofibrosis in the era of JAK inhibitors |
title | Treatment and management of myelofibrosis in the era of JAK inhibitors |
title_full | Treatment and management of myelofibrosis in the era of JAK inhibitors |
title_fullStr | Treatment and management of myelofibrosis in the era of JAK inhibitors |
title_full_unstemmed | Treatment and management of myelofibrosis in the era of JAK inhibitors |
title_short | Treatment and management of myelofibrosis in the era of JAK inhibitors |
title_sort | treatment and management of myelofibrosis in the era of jak inhibitors |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3753053/ https://www.ncbi.nlm.nih.gov/pubmed/23990704 http://dx.doi.org/10.2147/BTT.S34942 |
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