Myeloproliferative neoplasms
The BCR::ABL1-negative MPNs: JAK2, CALR and MPL drivers, WHO 2022 criteria for PV, ET and myelofibrosis, thrombotic risk groups, venesection to haematocrit under 0.45, aspirin, cytoreduction choices, JAK inhibitors and transplant in myelofibrosis.
Tier 1Condition
Written with AI assistance and fact-checked against the sources below. For exam study, not clinical decisions. How notes are made
At a glance
- Clonal stem cell disorders with overproduction of mature myeloid cells. The classical BCR::ABL1-negative MPNs are polycythaemia vera (PV), essential thrombocythaemia (ET) and primary myelofibrosis (PMF). CML is covered in Chronic leukaemia.
- Three driver mutations activate JAK-STAT signalling: JAK2, CALR and MPL. Finding one proves clonality.
- PV and ET are chronic. The main risks are arterial and venous thrombosis, then late progression to myelofibrosis or AML. Myelofibrosis shortens life.
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Sources
- The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Myeloid and Histiocytic/Dendritic NeoplasmsKhoury JD et al., Leukemia 2022;36:1703-19, 2022
- International Consensus Classification of myeloid neoplasms and acute leukemias: integrating morphologic, clinical, and genomic dataArber DA et al., Blood 2022;140:1200-28, 2022
- Philadelphia chromosome-negative classical myeloproliferative neoplasms: revised management recommendations from European LeukemiaNetBarbui T et al., Leukemia 2018;32:1057-69, 2018
- Appropriate management of polycythaemia vera with cytoreductive drug therapy: European LeukemiaNet 2021 recommendationsMarchetti M et al., Lancet Haematol 2022;9:e301-11, 2021
- A guideline for the diagnosis and management of polycythaemia vera. A British Society for Haematology GuidelineMcMullin MF et al., Br J Haematol 2019;184:176-91, 2019
- The management of myelofibrosis: A British Society for Haematology GuidelineMcLornan DP et al., Br J Haematol 2024, 2024
- Indication and management of allogeneic haematopoietic stem-cell transplantation in myelofibrosis: updated recommendations by the EBMT/ELN International Working GroupKröger N et al., Lancet Haematol 2024;11:e62-74, 2024
- Cardiovascular events and intensity of treatment in polycythemia vera (CYTO-PV)Marchioli R et al., N Engl J Med 2013;368:22-33, 2013
- Schedule of Pharmaceutical Benefits, November 2026 (ruxolitinib, fedratinib, momelotinib, hydroxycarbamide, peginterferon alfa-2a)AU/NZAustralian Government Department of Health, Disability and Ageing, November 2026
- Last updated
- 14 November 2026
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