The Korean journal of internal medicine

The Korean Association of Internal Medicine (대한내과학회)
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Guidelines for the management of myeloproliferative neoplasms

  • Choi, Chul Won (Division of Oncology-Hematology, Department of Internal Medicine, Korea University Guro Hospital) ;
  • Bang, Soo-Mee (Department of Internal Medicine, Seoul National University Bundang Hospital) ;
  • Jang, Seongsoo (Department of Laboratory Medicine, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Jung, Chul Won (Division of Hematology/Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Kim, Hee-Jin (Department of Laboratory Medicine & Genetics, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Kim, Ho Young (Department of Internal Medicine, Hallym University Sacred Heart Hospital) ;
  • Kim, Soo-Jeong (Department of Internal Medicine, Severance Hospital, Yonsei University College of Medicine) ;
  • Kim, Yeo-Kyeoung (Division of Hematology-Oncology, Department of Internal Medicine, Chonnam National University Hwasun Hospital) ;
  • Park, Jinny (Division of Hematology-Oncology, Department of Internal Medicine, Gachon University Gil Medical Center) ;
  • Won, Jong-Ho (Division of Hematology-Oncology, Department of Internal Medicine, Soonchunhyang University College of Medicine)
  • Received : 2015.01.07
  • Accepted : 2015.03.30
  • Published : 2015.11.01
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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 $JAK_2$ mutation is not only a standard criterion for diagnosis but is also a new target for drug development. The $JAK_{1/2}$ 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.

Keywords

References

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