Essential thrombocythemia: challenges in clinical practice and future prospects

[Display omitted] Essential thrombocythemia (ET) was first described in 1934, and subsequently, progress has been made in better understanding the molecular pathogenesis and which patients may have greatest risk of progression or vascular events. However, it has been more than a decade since a new therapy has been approved for ET. We are beginning to understand more comprehensively both the heterogeneity of this disease, which is largely driven by driver mutation status, as well as the effect of disease-related symptoms, such as fatigue, on patients. In this review we provide a practical overview of diagnosis and management of ET with focus on challenging patient scenarios and some consideration of what comprehensive care might entail. Finally, we also discuss newer therapies and how these might be assessed. Our knowledge about the biology of myeloproliferative neoplasms (MPNs) has exploded in the last 20 years, and this increased knowledge has led to advances in therapy. Introduced by Associate Editor Mario Cazzola, this Review Series brings readers up to date on our understanding of the natural history of the classical MPNs—polycythemia vera, essential thrombocythemia, and myelofibrosis—and the approaches to diagnosis, prognostication, and treatment for patients with these conditions.