Adolescent and young adult patients with ‘Philadelphia negative’ myeloproliferative neoplasms: a real world, UK tertiary-centre study

Abstract

Increasing numbers of adolescent and young adult (AYA) patients are diagnosed with myeloproliferative neoplasms (MPNs). Management strategies are often extrapolated from older MPN cohorts and frequently fail to address specific needs. Through retrospective description of 107 AYA patients registered at a UK specialist centre, we identify unique clinicopathological features compared with older patients, including lower incidence of JAK2 V617F and additional non-driver mutations, and a reduced rate of transformation. Despite these favourable disease characteristics, we demonstrate significant rates of venous thrombosis (>14 %). This data highlights areas of unmet clinical need and calls for tailored management approaches for AYA MPN patients.