Essential thrombocythaemia

Abstract

Synopsis Essential thrombocythaemia (ET) is a clonal stem cell disorder that frequently presents as an incidental finding of elevated platelet counts. Diagnosis depends on exclusion of other myeloproliferative disorders and reactive thrombocytosis. Patients with platelet counts above 1000 × 109/L should receive platelet‐lowering therapy, even if asymptomatic. Definitive recommendations cannot yet be made for asymptomatic patients with lower platelet counts. ET can be treated with alkylating agents, radioactive phosphorus or hydroxyurea, but there is evidence that these agents increase transformation to acute leukaemia. Interferon alfa‐2a and anagrelide are useful treatment agents, particularly in younger patients.

How common are myeloproliferative neoplasms? A systematic review and meta‐analysis

“…Due to the biological similarities these diseases possess with leukemias and myelodysplastic syndromes, disease misclassification may occur, misestimating the true incidence of these diseases. Thirdly, owing to the chronic nature of MPNs, individuals can be asymptomatic for many years , particularly those with PV and ET where diagnosis can be incidental . The reported incidence and prevalence rates are therefore likely to underestimate the true disease rate in the general population.…”

“…The MPNs have unknown etiology and are regarded as chronic diseases contributing significantly to morbidity and mortality . Signs and symptoms of these diseases generally do not present until later in life . Everyday living and quality of life (QOL) are affected by symptoms including fatigue, night sweats, and pruritus .…”