Prognosis

Developments in the management of primary myelofibrosis (PMF) have resulted in improved survival.[91][92]

Median overall survival (all ages) for patients with PMF was 4.0 years, based on registry data for 3689 PMF patients diagnosed between 2001 and 2015 (median follow-up 5.8 years).[93]​ Retrospective data from a single centre reported an improvement in median overall survival from 48 months (95% CI, 42-54 months) in patients diagnosed in between 2000 and 2010, to 63 months (95% CI, 55-71 months) in patients diagnosed between 2011 and 2020.[92] The study population comprised 844 patients; median age at new diagnosis of myelofibrosis was 66 years. Those treated with ruxolitinib had a median overall survival of 84 months (95% CI, 70-94 months).[92]

Among a cohort of 361 patients diagnosed with a myeloproliferative neoplasm between 1967 and 2017, median overall patient survival from time of diagnosis (stratified by age) was estimated to be:[94]

  • 20 years (age ≤40 years)

  • 8 years (age 41-60 years)

  • 3 years (age >60 years)

Adverse prognostic factors

Include age at onset (>64 years), anaemia (Hb <100 g/L [<10 g/dL]), constitutional symptoms, white blood cell count abnormalities (<4 ×10⁹/L or >12 × 10⁹/L [<4000/microlitre or >12,000/microlitre]), thrombocytopenia, circulating blast cells (>1%), and certain cytogenetic abnormalities (-5/del5q, -7/del7q, trisomy 8, 12p-).[11][43]

Mutations in CALR are associated with better overall survival compared with JAK2 V617F or MPL W515 mutations.[19]​ Type 1 CALR mutations (52-bp deletion) are more common, and have a more favourable impact on prognosis than type 2 CALR mutations (5-bp insertion) in PMF.[20][21]

Triple-negative mutation status (negative for JAK2, CALR, or MPL mutations) is associated with a worse prognosis in patients with PMF.[19][26]​​

ASXL1, EZH2, SRSF2, TP53, IDH1, IDH2, and U2AF1 mutations are considered high-molecular-risk mutations, associated with shorter overall and leukaemia-free survival.​[26][27]

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