Treatment and management of myelofibrosis in the era of JAK inhibitors

Clodagh Keohane, Deepti H Radia, Claire N HarrisonDepartment of Haematology, Guy's and St Thomas' NHS Foundation Trust, London, UKAbstract: Myelofibrosis (MF) can present as a primary disorder or evolve from polycythemia vera (PV) or essential thrombocythemia (ET) to post-PV MF or po...

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Autores principales:	Keohane C, Radia DH, Harrison CN
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2013
Materias:	Medicine (General) R5-920
Acceso en línea:	https://doaj.org/article/4f49a0ed1ad64993aad5401da2392057
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spelling	oai:doaj.org-article:4f49a0ed1ad64993aad5401da23920572021-12-02T08:40:19ZTreatment and management of myelofibrosis in the era of JAK inhibitors1177-54751177-5491https://doaj.org/article/4f49a0ed1ad64993aad5401da23920572013-08-01T00:00:00Zhttp://www.dovepress.com/treatment-and-management-of-myelofibrosis-in-the-era-of-jak-inhibitors-a14104https://doaj.org/toc/1177-5475https://doaj.org/toc/1177-5491Clodagh Keohane, Deepti H Radia, Claire N HarrisonDepartment of Haematology, Guy's and St Thomas' NHS Foundation Trust, London, UKAbstract: 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.Keywords: myelofibrosis, ruxolitinib, JAK inhibitorKeohane CRadia DHHarrison CNDove Medical PressarticleMedicine (General)R5-920ENBiologics: Targets & Therapy, Vol 2013, Iss default, Pp 189-198 (2013)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine (General) R5-920
spellingShingle	Medicine (General) R5-920 Keohane C Radia DH Harrison CN Treatment and management of myelofibrosis in the era of JAK inhibitors
description	Clodagh Keohane, Deepti H Radia, Claire N HarrisonDepartment of Haematology, Guy's and St Thomas' NHS Foundation Trust, London, UKAbstract: 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.Keywords: myelofibrosis, ruxolitinib, JAK inhibitor
format	article
author	Keohane C Radia DH Harrison CN
author_facet	Keohane C Radia DH Harrison CN
author_sort	Keohane C
title	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_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_sort	treatment and management of myelofibrosis in the era of jak inhibitors
publisher	Dove Medical Press
publishDate	2013
url	https://doaj.org/article/4f49a0ed1ad64993aad5401da2392057
work_keys_str_mv	AT keohanec treatmentandmanagementofmyelofibrosisintheeraofjakinhibitors AT radiadh treatmentandmanagementofmyelofibrosisintheeraofjakinhibitors AT harrisoncn treatmentandmanagementofmyelofibrosisintheeraofjakinhibitors
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Treatment and management of myelofibrosis in the era of JAK inhibitors

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