Assessing disease burden in patients with classic MPNs

• Published in: Best practice & research. Clinical haematology Vol. 27; no. 2; pp. 107 - 119
• Main Authors: Geyer, Holly, MD, Mesa, Ruben A., MD
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier Ltd 01-06-2014
• Subjects: Antineoplastic Agents - therapeutic use; Disease Management; Disease Progression; essential thrombocythemia; Hematology, Oncology and Palliative Medicine; Hemorrhage - diagnosis; Hemorrhage - drug therapy; Hemorrhage - etiology; Hemorrhage - pathology; Humans; MPN-SAF; myelofibrosis; myeloproliferative neoplasm; polycythemia vera; Polycythemia Vera - complications; Polycythemia Vera - diagnosis; Polycythemia Vera - drug therapy; Polycythemia Vera - pathology; Primary Myelofibrosis - complications; Primary Myelofibrosis - diagnosis; Primary Myelofibrosis - drug therapy; Primary Myelofibrosis - pathology; Prognosis; Risk; Severity of Illness Index; Splenomegaly - diagnosis; Splenomegaly - drug therapy; Splenomegaly - etiology; Splenomegaly - pathology; symptom; Thrombocythemia, Essential - complications; Thrombocythemia, Essential - diagnosis; Thrombocythemia, Essential - drug therapy; Thrombocythemia, Essential - pathology; Thrombosis - diagnosis; Thrombosis - drug therapy; Thrombosis - etiology; Thrombosis - pathology; Treatment Outcome; myelofibrosis; symptom; MPN-SAF; polycythemia vera; essential thrombocythemia; risk; myeloproliferative neoplasm
• ISSN: 1521-6926; 1532-1924
• DOI: 10.1016/j.beha.2014.07.006

Myeloproliferative neoplasm (MPN) disorders including polycythemia vera (PV), essential thrombocythemia (ET) and myelofibrosis (MF), are recognized amongst the world of malignancies for their unique disease–burden profiles. Symptom management remains a prime directive for all MPN disorders. Limited by the dramatic heterogeneity and disparate severity amongst symptoms, only recently have researchers possessed the scoring tools necessary to quantify the MPN symptom burden and investigate its role in patient prognosis. In addition to symptom management, clinicians are also tasked with managing the numerous complications that arise from MPN progression including splenomegaly, cytopenias, thrombotic and hemorrhagic events and transformation to MF (from PV or ET) or acute myelogenous leukemia. In this article, we discuss the pleiotropic solidarity of the MPN symptom profile, inherent complications that define the disorders, available patient-reported outcome tools, the role of risk-scoring algorithms and open arenas for ongoing MPN symptom research.