Description of recurrent genetic abnormalities in driver genes, a better appreciation of the key diagnostic role of bone marrow features, results of large epidemiologic studies, and landmark controlled clinical trials have resulted in a reappraisal of the approach to polycythemia vera and essential thrombocythemia, authors noted in a recent review.

The revised 2017 WHO classification of polycythemia vera and essential thrombocythemia allows early diagnosis and accurate distinction from other chronic myeloproliferative neoplasms, particularly prefibrotic myelofibrosis. The prognostic value of selected mutations is being investigated and JAK2V617F mutation is currently incorporated as risk variable in prognostic system for essential thrombocythemia. Risk-adjusted stratification is used to select therapeutic approaches that include target agents.