The JAK2V617F mutation and the role of therapeutic agents in alleviating myeloproliferative neoplasm symptom burden

Lai Yee Orbell; Nouf Abutheraa; Andrew S. Duncombe; Mary Frances McMullin; Ruben Mesa; Charlene McShane; Glen James; Lesley Anderson

doi:10.1002/jha2.805

The JAK2V617F mutation and the role of therapeutic agents in alleviating myeloproliferative neoplasm symptom burden

Lai Yee Orbell, Nouf Abutheraa, Andrew S. Duncombe, Mary Frances McMullin, Ruben Mesa, Charlene McShane, Glen James, Lesley Anderson^* (Corresponding Author)

^*Corresponding author for this work

Research output: Contribution to journal › Article › peer-review

Abstract

Alleviating symptom burden in patients with myeloproliferative neoplasms (MPNs) is imperative to achieving optimal management. Research remains to elucidate the relationship between the JAK2V617F (Janus kinase 2) mutation present in many MPN patients, and the symptomatology they experience. This retrospective study analysed data collected from MPN patients included in the Myeloproliferative Neoplasms: An In‐depth Case–Control (MOSAICC) pilot study. The MPN Symptom Assessment Form was administered, and median symptom scores were compared between JAK2V617F‐positive and JAK2V617F‐negative groups. Multivariate logistic regression analysis adjusted for confounding variables. Overall, 106 MPN patients participated: 65.1% were JAK2V617F positive, 30.2% were JAK2V617F negative and 4.7% had an unknown status. Multivariate analysis revealed a low symptom burden for early satiety (p < 0.01), dizziness (p < 0.05), cough (p < 0.05) and bone pain (p < 0.01) in those receiving venesection alone. Interferon alpha was significantly associated (p < 0.05) with severe burden for 16 of the 27 symptoms. JAK2V617F‐positive females experienced a greater symptom burden than JAK2V617F‐positive males. There was no discernible relationship between the JAK2V617F mutation and symptom burden in MPN patients, unlike the therapeutic agents investigated. Larger studies are required to validate these results and identify mechanisms of symptom development and control in MPN patients.

Original language	English
Pages (from-to)	1071-1080
Number of pages	10
Journal	European Journal of Haematology
Volume	4
Issue number	4
Early online date	31 Oct 2023
DOIs	https://doi.org/10.1002/jha2.805
Publication status	Published - Nov 2023

Bibliographical note

Funding information:
MPNVoice,Grant/AwardNumber:0001

Acknowledgements:
The authors would like to express gratitude to the participants who gave their time to take part in the MOSAICC pilot study. We would also like to acknowledge the work of the charity MPN Voice (grant number 0001), formerly MPD Voice, who kindly funded the work of the MOSAICC pilot study. Finally, we would like to thank Dr Barry Crouch from the University of Aberdeen Digital Research Service, whose knowledge and assistance in coding and statistical software were fundamental to the completion of this research.

Data Availability Statement

Data access can be sought through submission of a data request to the MOSAICC steering group led by Prof Anderson (lesley.anderson@abdn.ac.uk). If approved a data transfer arrangement would be required.

Keywords

essential thrombocythaemia
Janus kinase 2
management
myeloproliferative neoplasm
Polycythaemia vera
primary myelofibrosis
treatment

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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Cite this

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title = "The JAK2V617F mutation and the role of therapeutic agents in alleviating myeloproliferative neoplasm symptom burden",

abstract = "Alleviating symptom burden in patients with myeloproliferative neoplasms (MPNs) is imperative to achieving optimal management. Research remains to elucidate the relationship between the JAK2V617F (Janus kinase 2) mutation present in many MPN patients, and the symptomatology they experience. This retrospective study analysed data collected from MPN patients included in the Myeloproliferative Neoplasms: An In‐depth Case–Control (MOSAICC) pilot study. The MPN Symptom Assessment Form was administered, and median symptom scores were compared between JAK2V617F‐positive and JAK2V617F‐negative groups. Multivariate logistic regression analysis adjusted for confounding variables. Overall, 106 MPN patients participated: 65.1% were JAK2V617F positive, 30.2% were JAK2V617F negative and 4.7% had an unknown status. Multivariate analysis revealed a low symptom burden for early satiety (p < 0.01), dizziness (p < 0.05), cough (p < 0.05) and bone pain (p < 0.01) in those receiving venesection alone. Interferon alpha was significantly associated (p < 0.05) with severe burden for 16 of the 27 symptoms. JAK2V617F‐positive females experienced a greater symptom burden than JAK2V617F‐positive males. There was no discernible relationship between the JAK2V617F mutation and symptom burden in MPN patients, unlike the therapeutic agents investigated. Larger studies are required to validate these results and identify mechanisms of symptom development and control in MPN patients.",

keywords = "essential thrombocythaemia, Janus kinase 2, management, myeloproliferative neoplasm, Polycythaemia vera, primary myelofibrosis, treatment",

author = "Orbell, {Lai Yee} and Nouf Abutheraa and Duncombe, {Andrew S.} and McMullin, {Mary Frances} and Ruben Mesa and Charlene McShane and Glen James and Lesley Anderson",

note = "Funding information: MPNVoice,Grant/AwardNumber:0001 Acknowledgements: The authors would like to express gratitude to the participants who gave their time to take part in the MOSAICC pilot study. We would also like to acknowledge the work of the charity MPN Voice (grant number 0001), formerly MPD Voice, who kindly funded the work of the MOSAICC pilot study. Finally, we would like to thank Dr Barry Crouch from the University of Aberdeen Digital Research Service, whose knowledge and assistance in coding and statistical software were fundamental to the completion of this research.",

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doi = "10.1002/jha2.805",

language = "English",

volume = "4",

pages = "1071--1080",

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T1 - The JAK2V617F mutation and the role of therapeutic agents in alleviating myeloproliferative neoplasm symptom burden

AU - Orbell, Lai Yee

AU - Abutheraa, Nouf

AU - Duncombe, Andrew S.

AU - McMullin, Mary Frances

AU - Mesa, Ruben

AU - McShane, Charlene

AU - James, Glen

AU - Anderson, Lesley

N1 - Funding information: MPNVoice,Grant/AwardNumber:0001 Acknowledgements: The authors would like to express gratitude to the participants who gave their time to take part in the MOSAICC pilot study. We would also like to acknowledge the work of the charity MPN Voice (grant number 0001), formerly MPD Voice, who kindly funded the work of the MOSAICC pilot study. Finally, we would like to thank Dr Barry Crouch from the University of Aberdeen Digital Research Service, whose knowledge and assistance in coding and statistical software were fundamental to the completion of this research.

PY - 2023/11

Y1 - 2023/11

N2 - Alleviating symptom burden in patients with myeloproliferative neoplasms (MPNs) is imperative to achieving optimal management. Research remains to elucidate the relationship between the JAK2V617F (Janus kinase 2) mutation present in many MPN patients, and the symptomatology they experience. This retrospective study analysed data collected from MPN patients included in the Myeloproliferative Neoplasms: An In‐depth Case–Control (MOSAICC) pilot study. The MPN Symptom Assessment Form was administered, and median symptom scores were compared between JAK2V617F‐positive and JAK2V617F‐negative groups. Multivariate logistic regression analysis adjusted for confounding variables. Overall, 106 MPN patients participated: 65.1% were JAK2V617F positive, 30.2% were JAK2V617F negative and 4.7% had an unknown status. Multivariate analysis revealed a low symptom burden for early satiety (p < 0.01), dizziness (p < 0.05), cough (p < 0.05) and bone pain (p < 0.01) in those receiving venesection alone. Interferon alpha was significantly associated (p < 0.05) with severe burden for 16 of the 27 symptoms. JAK2V617F‐positive females experienced a greater symptom burden than JAK2V617F‐positive males. There was no discernible relationship between the JAK2V617F mutation and symptom burden in MPN patients, unlike the therapeutic agents investigated. Larger studies are required to validate these results and identify mechanisms of symptom development and control in MPN patients.

AB - Alleviating symptom burden in patients with myeloproliferative neoplasms (MPNs) is imperative to achieving optimal management. Research remains to elucidate the relationship between the JAK2V617F (Janus kinase 2) mutation present in many MPN patients, and the symptomatology they experience. This retrospective study analysed data collected from MPN patients included in the Myeloproliferative Neoplasms: An In‐depth Case–Control (MOSAICC) pilot study. The MPN Symptom Assessment Form was administered, and median symptom scores were compared between JAK2V617F‐positive and JAK2V617F‐negative groups. Multivariate logistic regression analysis adjusted for confounding variables. Overall, 106 MPN patients participated: 65.1% were JAK2V617F positive, 30.2% were JAK2V617F negative and 4.7% had an unknown status. Multivariate analysis revealed a low symptom burden for early satiety (p < 0.01), dizziness (p < 0.05), cough (p < 0.05) and bone pain (p < 0.01) in those receiving venesection alone. Interferon alpha was significantly associated (p < 0.05) with severe burden for 16 of the 27 symptoms. JAK2V617F‐positive females experienced a greater symptom burden than JAK2V617F‐positive males. There was no discernible relationship between the JAK2V617F mutation and symptom burden in MPN patients, unlike the therapeutic agents investigated. Larger studies are required to validate these results and identify mechanisms of symptom development and control in MPN patients.

KW - essential thrombocythaemia

KW - Janus kinase 2

KW - management

KW - myeloproliferative neoplasm

KW - Polycythaemia vera

KW - primary myelofibrosis

KW - treatment

U2 - 10.1002/jha2.805

DO - 10.1002/jha2.805

M3 - Article

C2 - 38024634

SN - 0902-4441

VL - 4

SP - 1071

EP - 1080

JO - European Journal of Haematology

JF - European Journal of Haematology

IS - 4

ER -

The JAK2V617F mutation and the role of therapeutic agents in alleviating myeloproliferative neoplasm symptom burden

Abstract

Bibliographical note

Data Availability Statement

Keywords

UN SDGs

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