Korean J Intern Med.  2021 Jan;36(1):45-62. 10.3904/kjim.2020.319.

The 2020 revision of the guidelines for the management of myeloproliferative neoplasms

Affiliations
  • 1Division of Hematology, Department of Internal Medicine, Konkuk University Medical Center, Seoul, Korea
  • 2Department of Internal Medicine, Daegu Catholic University School of Medicine, Daegu, Korea
  • 3Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Korea
  • 4Department of Hematology, Seoul St. Mary’s Hematology Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
  • 5Division of Hematology and Medical Oncology, Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea
  • 6Department of Laboratory Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
  • 7Division of Hematology/Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
  • 8Department of Laboratory Medicine & Genetics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
  • 9Department of Internal Medicine, Hallym University Medical Center, Anyang, Korea
  • 10Department of Internal Medicine, Yeungnam University College of Medicine, Daegu, Korea
  • 11Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea
  • 12Department of Internal Medicine, Ewha Womans University School of Medicine, Seoul, Korea
  • 13Department of Internal Medicine, Veterans Health Service Medical Center, Seoul, Korea
  • 14Division of Hematology, Department of Internal Medicine, Gachon University Gil Medical Center, Incheon, Korea
  • 15Division of Hematology-Oncology, Department of Internal Medicine, Soonchunhyang University College of Medicine, Seoul, Korea
  • 16Division of Hematology-Oncology, Department of Internal Medicine, Korea University Guro Hospital, Seoul, Korea

Abstract

In 2016, the World Health Organization revised the diagnostic criteria for myeloproliferative neoplasms (MPNs) based on the discovery of disease-driving genetic aberrations and extensive analysis of the clinical characteristics of patients with MPNs. Recent studies have suggested that additional somatic mutations have a clinical impact on the prognosis of patients harboring these genetic abnormalities. Treatment strategies have also advanced with the introduction of JAK inhibitors, one of which has been approved for the treatment of patients with myelofibrosis and those with hydroxyurea-resistant or intolerant polycythemia vera. Recently developed drugs aim to elicit hematologic responses, as well as symptomatic and molecular responses, and the response criteria were refined accordingly. Based on these changes, we have revised the guidelines and present the diagnosis, treatment, and risk stratification of MPNs encountered in Korea.

Keyword

Polycythemia vera; Thrombocythemia; essential; Primary myelofibrosis; Practice guideline
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