Addressing symptom burden in myeloproliferative neoplasms

Abstract

Patients with myeloproliferative neoplasms (MPNs) suffer from often debilitating constitutional symptoms that negatively impact quality of life to a degree similar to patients with metastatic solid tumors. Despite heterogeneity in the breadth and severity of symptoms in MPNs, research into symptom assessment has led to the creation of well validated patient reported outcome tools, including the Myeloproliferative Neoplasm Symptom Assessment Form Total Symptom Score. Currently available pharmacologic therapies, particularly JAK inhibitors, result in substantial reduction in symptom burden for patients with myelofibrosis, as well as select patient with polycythemia vera and essential thrombocythemia. Non-pharmacologic therapies including yoga and meditation have also been investigated. In this review, we focus on the pathogenesis and assessment of constitutional symptoms in MPNs. We detail currently available therapies to address symptom burden and highlight several novel agents in development. We end by discussing unmet needs and exploring the future of symptom assessment and treatment in MPNs.

Keywords: JAK inhibitor; Myelofibrosis; Quality of life; Systemic symptoms.