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

Research output: Contribution to journalArticlepeer-review

11 Citations (Scopus)

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 languageEnglish
Pages (from-to)792-797
Number of pages6
JournalBritish Journal of Haematology
Volume193
Issue number4
Early online date9 Mar 2021
DOIs
Publication statusPublished - May 2021

Keywords

  • clinical data
  • genomics
  • myeloproliferative neoplasms-unclassifiable
  • outcome

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