title: Guidelines for the management of myeloproliferative neoplasms.
abstract: Polycythemia vera, essential thrombocythemia, and primary myelofibrosis are collectively known as 'Philadelphia-negative classical myeloproliferative neoplasms (MPNs).' The discovery of new genetic aberrations such as Janus kinase 2 (JAK2) have enhanced our understanding of the pathophysiology of MPNs. Currently, the JAK2 mutation is not only a standard criterion for diagnosis but is also a new target for drug development. The JAK1/2 inhibitor, ruxolitinib, was the first JAK inhibitor approved for patients with intermediate- to high-risk myelofibrosis and its effects in improving symptoms and survival benefits were demonstrated by randomized controlled trials. In 2011, the Korean Society of Hematology MPN Working Party devised diagnostic and therapeutic guidelines for Korean MPN patients. Subsequently, other genetic mutations have been discovered and many kinds of new drugs are now under clinical investigation. In view of recent developments, we have revised the guidelines for the diagnosis and management of MPN based on published evidence and the experiences of the expert panel. Here we describe the epidemiology, new genetic mutations, and novel therapeutic options as well as diagnostic criteria and standard treatment strategies for MPN patients in Korea.
authors.affiliation: Division of Oncology-Hematology, Department of Internal Medicine, Korea University Guro Hospital, Seoul, Korea.
authors.forename: Chul Won
authors.initials: CW
authors.lastname: Choi
authors.affiliation: Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Korea.
authors.forename: Soo-Mee
authors.initials: SM
authors.lastname: Bang
authors.affiliation: Department of Laboratory Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
authors.forename: Seongsoo
authors.initials: S
authors.lastname: Jang
authors.affiliation: Division of Hematology/Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors.forename: Chul Won
authors.initials: CW
authors.lastname: Jung
authors.affiliation: Department of Laboratory Medicine & Genetics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors.forename: Hee-Jin
authors.initials: HJ
authors.lastname: Kim
authors.affiliation: Department of Internal Medicine, Hallym University Sacred Heart Hospital, Anyang, Korea.
authors.forename: Ho Young
authors.initials: HY
authors.lastname: Kim
authors.affiliation: Department of Internal Medicine, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Soo-Jeong
authors.initials: SJ
authors.lastname: Kim
authors.affiliation: Division of Hematology-Oncology, Department of Internal Medicine, Chonnam National University Hwasun Hospital, Hwasun, Korea.
authors.forename: Yeo-Kyeoung
authors.initials: YK
authors.lastname: Kim
authors.affiliation: Division of Hematology-Oncology, Department of Internal Medicine, Gachon University Gil Medical Center, Incheon, Korea.
authors.forename: Jinny
authors.initials: J
authors.lastname: Park
authors.affiliation: Division of Hematology-Oncology, Department of Internal Medicine, Soonchunhyang University College of Medicine, Seoul, Korea.
authors.forename: Jong-Ho
authors.initials: JH
authors.lastname: Won
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use*; D044466:Asian People / Q000235:genetics; D006801:Humans; D053614:Janus Kinase 2 / Q000037:antagonists & inhibitors* / Q000235:genetics / Q000378:metabolism; D058990:Molecular Targeted Therapy; D009154:Mutation; D009196:Myeloproliferative Disorders / Q000175:diagnosis / Q000188:drug therapy / Q000201:enzymology / Q000208:ethnology / Q000235:genetics; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use*; D056910:Republic of Korea / Q000453:epidemiology; D012307:Risk Factors; D015398:Signal Transduction / Q000187:drug effects; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents; D047428:Protein Kinase Inhibitors; C507924:JAK2 protein, human; D053614:Janus Kinase 2
keywords: Polycythemia vera; Practice guideline; Primary myelofibrosis; Thrombocythemia, essential