Prognosis

Life expectancy of patients with essential thrombocythaemia (ET) is reported to be modestly reduced compared with that of the general population. Median overall survival (stratified by age) has been estimated to be:[68]

  • 35 years (age ≤40 years)

  • 22 years (age 41 to 60 years)

  • 11 years (age >60 years).

Median overall survival (all aged) for patients with ET was 12.1 years in one study, based on registry data for 8,768 patients diagnosed between 2001 and 2015 (median follow-up 5.8 years). During the study period, 134 (1.5%) ET patients progressed to acute myeloid leukaemia (AML); median time to AML transformation was 5.8 years.[69]

Development of either AML or myelofibrosis is uncommon. The 15-year cumulative risk of transformation to AML or myelofibrosis has been reported to be in the range of 2% to 5.3% and 4% to 11%, respectively.[70][71][72]

Janus kinase 2 (JAK2), calreticulin (CALR), or myeloproliferative leukaemia virus oncogene (MPL) mutation status does not affect survival in patients with ET.[39]​ However, JAK2 and MPL mutations have been associated with higher risk of arterial thrombosis. MPL mutations have also been associated with fibrotic progression.

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