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November 6, 2023British Journal of HaematologyOpen Access

Diagnosis and evaluation of prognosis of myelofibrosis: A British Society for Haematology Guideline

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Authors

DMDonal P. McLornanAGAnna L. GodfreyAGAnna Green

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Overview

Clinical guideline outlines diagnostic and prognostic frameworks in myelofibrosis, highlighting molecular stratification and integrated pathology.

Key Points

  • To update evidence-based clinical recommendations from the British Society for Haematology on the diagnosis and prognostic assessment of primary, post-essential thrombocythaemia, post-polycythaemia vera, and prefibrotic myelofibrosis.
  • Literature searches were conducted across Medline, PubMed, and Cochrane covering human clinical studies, trials, and meta-analyses published from 2012 to mid-2022.
  • Evidence quality and recommendation strength were appraised using GRADE criteria, with review by the BSH Guidelines Committee, patient representatives, and external international experts.
  • Bone marrow evaluation requiring reticulin grading (minimum grade 2) alongside screening for driver mutations (JAK2, CALR, MPL) forms the core diagnostic requirement to differentiate myelofibrosis from mimics.
  • Comprehensive myeloid next-generation sequencing panels covering high molecular risk genes (e.g., ASXL1, SRSF2, EZH2, IDH1/2) and cytogenetic testing are recommended to refine risk in transplant-eligible and high-risk cohorts.
  • Prognostic models—including IPSS, DIPSS-plus, MIPSS70+ v2.0, MYSEC-PM, and personalised calculators—must be dynamically applied at regular intervals to guide therapy and transplant planning.

Cite This Study

McLornan et al. (2023) studied this question.

synapsesocial.com/papers/6a1027ea5725bbd5cc609639https://doi.org/10.1111/bjh.19164
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