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The 2025 ONKOPEDIA guideline on myelofibrosis (MF), providing an updated, evidence-based framework for the diagnosis and management of MF, was published in the International Journal of Cancer by Griesshammer et al. The guideline was developed by an expert panel nominated by the German Society for Hematology and Oncology (DGHO).
Key data: Standardized patient-reported symptom evaluation using instruments such as the Myeloproliferative Neoplasm Symptom Assessment Form Total Symptom Score (MPN-SAF TSS) is recommended. For prefibrotic primary MF (pre-PMF), the importance of precise diagnosis, symptom assessment, molecularly informed risk stratification, and individualized therapeutic decision-making is emphasized. Molecular risk models, including the Mutation-Enhanced, Karyotype-Enhanced International Prognostic Scoring System Version 2.0 for patients aged ≤70 years (MIPSS70+ v2.0) for PMF and the Myelofibrosis Secondary to Polycythemia Vera (PV) and Essential Thrombocythemia (ET) Prognostic Model (MYSEC-PM) for secondary MF, are recommended. Janus kinase inhibitors (JAKi) are identified as the standard of care for symptomatic MF, though JAKi selection should be stratified according to the patient’s clinical profile; patients should also be considered for clinical trial enrollment when appropriate. Ruxolitinib treatment response should be assessed longitudinally, with the Ruxolitinib Response at 6 months (RR6) score recommended to help identify treatment failure.
Key learning: These guideline recommendations reinforce a patient-centered, molecularly informed approach to MF management, emphasizing symptom-driven decision-making, early identification of treatment failure, and early referral for transplantation.
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