Korean J Intern Med.
2015 Nov;30(6):771-788. 10.3904/kjim.2015.30.6.771.
Guidelines for the management of myeloproliferative neoplasms
- Affiliations
-
- 1Division of Oncology-Hematology, Department of Internal Medicine, Korea University Guro Hospital, Seoul, Korea.
- 2Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Korea.
- 3Department of Laboratory Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
- 4Division of Hematology/Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
- 5Department of Laboratory Medicine & Genetics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
- 6Department of Internal Medicine, Hallym University Sacred Heart Hospital, Anyang, Korea.
- 7Department of Internal Medicine, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.
- 8Division of Hematology-Oncology, Department of Internal Medicine, Chonnam National University Hwasun Hospital, Hwasun, Korea.
- 9Division of Hematology-Oncology, Department of Internal Medicine, Gachon University Gil Medical Center, Incheon, Korea.
- 10Division of Hematology-Oncology, Department of Internal Medicine, Soonchunhyang University College of Medicine, Seoul, Korea. jhwon@schmc.ac.kr
- KMID: 2381581
- DOI: http://doi.org/10.3904/kjim.2015.30.6.771
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.
MeSH Terms
-
Antineoplastic Agents/*therapeutic use
Asian Continental Ancestry Group/genetics
Humans
Janus Kinase 2/*antagonists & inhibitors/genetics/metabolism
Molecular Targeted Therapy
Mutation
Myeloproliferative Disorders/diagnosis/drug therapy/enzymology/ethnology/genetics
Protein Kinase Inhibitors/*therapeutic use
Republic of Korea/epidemiology
Risk Factors
Signal Transduction/drug effects
Treatment Outcome
Antineoplastic Agents
Janus Kinase 2
Protein Kinase Inhibitors
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