Clinicopathological characterisation of myeloproliferative neoplasm-unclassifiable (MPN-U): a retrospective analysis from a large UK tertiary referral centre

Paul Deschamps; Mufaddal Moonim; Deepti Radia; Natalia Curto-Garcia; Claire Woodley; Sarah Bassiony; Jennifer O'Sullivan; Patrick Harrington; Kavita Raj; Yvonne Francis; Shahram Kordasti; Sahra Ali; Claire N. Harrison; Donal P. McLornan

doi:10.1111/bjh.17375

Clinicopathological characterisation of myeloproliferative neoplasm-unclassifiable (MPN-U): a retrospective analysis from a large UK tertiary referral centre

Paul Deschamps^*, Mufaddal Moonim, Deepti Radia, Natalia Curto-Garcia, Claire Woodley, Sarah Bassiony, Jennifer O'Sullivan, Patrick Harrington, Kavita Raj, Yvonne Francis, Shahram Kordasti, Sahra Ali, Claire N. Harrison, Donal P. McLornan

^*Corresponding author for this work

Comprehensive Cancer Centre

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

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Abstract

Myeloproliferative neoplasm-unclassifiable (MPN-U) presents an MPN-type phenotype that fails to meet diagnostic criteria for other MPN variants. Variability in the clinicopathological phenotypes presents many challenges. Amongst a registry cohort of 1512 patients with MPN, 82 with MPN-U were included, with a median (range) age of 49·7 (13–79) years. Albeit heterogeneous, common presentation features included raised lactate dehydrogenase, thrombocytosis and clustered/pleomorphic megakaryocytes on trephine biopsy. Thrombosis was common (21%), necessitating vigilance. The median event-free survival was 11·25 years (95% confidence interval 9·3–not reached), significantly shortened in cases with lower platelet counts (<500 × 10 ⁹/l) and a leucocytosis (≥12 × 10 ⁹/l) at presentation. Generation of potential MPN-U prognostic scores is required.

Original language	English
Pages (from-to)	792-797
Number of pages	6
Journal	British Journal of Haematology
Volume	193
Issue number	4
Early online date	9 Mar 2021
DOIs	https://doi.org/10.1111/bjh.17375
Publication status	Published - May 2021

Keywords

clinical data
genomics
myeloproliferative neoplasms-unclassifiable
outcome

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10.1111/bjh.17375

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Deschamps, P., Moonim, M., Radia, D., Curto-Garcia, N., Woodley, C., Bassiony, S., O'Sullivan, J., Harrington, P., Raj, K., Francis, Y., Kordasti, S., Ali, S., Harrison, C. N., & McLornan, D. P. (2021). Clinicopathological characterisation of myeloproliferative neoplasm-unclassifiable (MPN-U): a retrospective analysis from a large UK tertiary referral centre. British Journal of Haematology, 193(4), 792-797. https://doi.org/10.1111/bjh.17375

@article{899af8dce7f248ccab6a969fb4e22033,

title = "Clinicopathological characterisation of myeloproliferative neoplasm-unclassifiable (MPN-U): a retrospective analysis from a large UK tertiary referral centre",

abstract = "Myeloproliferative neoplasm-unclassifiable (MPN-U) presents an MPN-type phenotype that fails to meet diagnostic criteria for other MPN variants. Variability in the clinicopathological phenotypes presents many challenges. Amongst a registry cohort of 1512 patients with MPN, 82 with MPN-U were included, with a median (range) age of 49·7 (13–79) years. Albeit heterogeneous, common presentation features included raised lactate dehydrogenase, thrombocytosis and clustered/pleomorphic megakaryocytes on trephine biopsy. Thrombosis was common (21%), necessitating vigilance. The median event-free survival was 11·25 years (95% confidence interval 9·3–not reached), significantly shortened in cases with lower platelet counts (<500 × 10 9/l) and a leucocytosis (≥12 × 10 9/l) at presentation. Generation of potential MPN-U prognostic scores is required. ",

keywords = "clinical data, genomics, myeloproliferative neoplasms-unclassifiable, outcome",

author = "Paul Deschamps and Mufaddal Moonim and Deepti Radia and Natalia Curto-Garcia and Claire Woodley and Sarah Bassiony and Jennifer O'Sullivan and Patrick Harrington and Kavita Raj and Yvonne Francis and Shahram Kordasti and Sahra Ali and Harrison, {Claire N.} and McLornan, {Donal P.}",

note = "Funding Information: Paul Deschamps, Claire N. Harrison and Donal P. McLornan performed the study design, analysis and were involved in drafting the paper. All other co-authors contributed data, critically revised the paper and approved the submitted version. Publisher Copyright: {\textcopyright} 2021 British Society for Haematology and John Wiley & Sons Ltd Copyright: Copyright 2021 Elsevier B.V., All rights reserved.",

year = "2021",

month = may,

doi = "10.1111/bjh.17375",

language = "English",

volume = "193",

pages = "792--797",

journal = "British Journal of Haematology",

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Deschamps, P, Moonim, M, Radia, D, Curto-Garcia, N, Woodley, C, Bassiony, S, O'Sullivan, J, Harrington, P, Raj, K, Francis, Y, Kordasti, S, Ali, S, Harrison, CN & McLornan, DP 2021, 'Clinicopathological characterisation of myeloproliferative neoplasm-unclassifiable (MPN-U): a retrospective analysis from a large UK tertiary referral centre', British Journal of Haematology, vol. 193, no. 4, pp. 792-797. https://doi.org/10.1111/bjh.17375

TY - JOUR

T1 - Clinicopathological characterisation of myeloproliferative neoplasm-unclassifiable (MPN-U)

T2 - a retrospective analysis from a large UK tertiary referral centre

AU - Deschamps, Paul

AU - Moonim, Mufaddal

AU - Radia, Deepti

AU - Curto-Garcia, Natalia

AU - Woodley, Claire

AU - Bassiony, Sarah

AU - O'Sullivan, Jennifer

AU - Harrington, Patrick

AU - Raj, Kavita

AU - Francis, Yvonne

AU - Kordasti, Shahram

AU - Ali, Sahra

AU - Harrison, Claire N.

AU - McLornan, Donal P.

N1 - Funding Information: Paul Deschamps, Claire N. Harrison and Donal P. McLornan performed the study design, analysis and were involved in drafting the paper. All other co-authors contributed data, critically revised the paper and approved the submitted version. Publisher Copyright: © 2021 British Society for Haematology and John Wiley & Sons Ltd Copyright: Copyright 2021 Elsevier B.V., All rights reserved.

PY - 2021/5

Y1 - 2021/5

N2 - Myeloproliferative neoplasm-unclassifiable (MPN-U) presents an MPN-type phenotype that fails to meet diagnostic criteria for other MPN variants. Variability in the clinicopathological phenotypes presents many challenges. Amongst a registry cohort of 1512 patients with MPN, 82 with MPN-U were included, with a median (range) age of 49·7 (13–79) years. Albeit heterogeneous, common presentation features included raised lactate dehydrogenase, thrombocytosis and clustered/pleomorphic megakaryocytes on trephine biopsy. Thrombosis was common (21%), necessitating vigilance. The median event-free survival was 11·25 years (95% confidence interval 9·3–not reached), significantly shortened in cases with lower platelet counts (<500 × 10 9/l) and a leucocytosis (≥12 × 10 9/l) at presentation. Generation of potential MPN-U prognostic scores is required.

AB - Myeloproliferative neoplasm-unclassifiable (MPN-U) presents an MPN-type phenotype that fails to meet diagnostic criteria for other MPN variants. Variability in the clinicopathological phenotypes presents many challenges. Amongst a registry cohort of 1512 patients with MPN, 82 with MPN-U were included, with a median (range) age of 49·7 (13–79) years. Albeit heterogeneous, common presentation features included raised lactate dehydrogenase, thrombocytosis and clustered/pleomorphic megakaryocytes on trephine biopsy. Thrombosis was common (21%), necessitating vigilance. The median event-free survival was 11·25 years (95% confidence interval 9·3–not reached), significantly shortened in cases with lower platelet counts (<500 × 10 9/l) and a leucocytosis (≥12 × 10 9/l) at presentation. Generation of potential MPN-U prognostic scores is required.

KW - clinical data

KW - genomics

KW - myeloproliferative neoplasms-unclassifiable

KW - outcome

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U2 - 10.1111/bjh.17375

DO - 10.1111/bjh.17375

M3 - Article

C2 - 33751548

AN - SCOPUS:85102281008

SN - 0007-1048

VL - 193

SP - 792

EP - 797

JO - British Journal of Haematology

JF - British Journal of Haematology

IS - 4

ER -

Clinicopathological characterisation of myeloproliferative neoplasm-unclassifiable (MPN-U): a retrospective analysis from a large UK tertiary referral centre

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Keywords

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