• 1 Department of Medicine, LKS Faculty of Medicine, School of Clinical Medicine, The University of Hong Kong, Pok Fu Lam, Hong Kong, China.
  • 2 Department of Medicine, LKS Faculty of Medicine, School of Clinical Medicine, The University of Hong Kong, Pok Fu Lam, Hong Kong, China
  • 3 Department of Hematology-Oncology, National University Cancer Institute, Singapore, Singapore
  • 4 Department of Medicine, Sunway Medical Centre, Shah Alam, Selangor, Malaysia
  • 5 Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea
  • 6 Department of Haematology, Singapore General Hospital, Singapore, Singapore
  • 7 King Chulalongkorn Memorial Hospital, Chulalongkorn University, Bangkok, Thailand
  • 8 University of Santo Tomas Hospital PH, Manila, Philippines
  • 9 Blood Disease Hospital and Institute of Hematology, Chinese Academy of Medical Sciences Peking Union Medical College, Tianjin, China
  • 10 Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan
  • 11 Chang Gung Memorial Hospital-Linkou, Chang Gung University, Taoyuan, Taiwan
  • 12 University of Malaya, Kuala Lumpur, Malaysia
  • 13 Department of Laboratory Medicine, National Taiwan University Hospital, Taipei, Taiwan
Clin Exp Med, 2023 Sep 25.
PMID: 37747591 DOI: 10.1007/s10238-023-01189-9


Myeloproliferative neoplasms (MPN) are a heterogeneous group of clonal hematopoietic stem cell disorders characterized clinically by the proliferation of one or more hematopoietic lineage(s). The classical Philadelphia-chromosome (Ph)-negative MPNs include polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF). The Asian Myeloid Working Group (AMWG) comprises representatives from fifteen Asian centers experienced in the management of MPN. This consensus from the AMWG aims to review the current evidence in the risk stratification and treatment of Ph-negative MPN, to identify management gaps for future improvement, and to offer pragmatic approaches for treatment commensurate with different levels of resources, drug availabilities and reimbursement policies in its constituent regions. The management of MPN should be patient-specific and based on accurate diagnostic and prognostic tools. In patients with PV, ET and early/prefibrotic PMF, symptoms and risk stratification will guide the need for early cytoreduction. In younger patients requiring cytoreduction and in those experiencing resistance or intolerance to hydroxyurea, recombinant interferon-α preparations (pegylated interferon-α 2A or ropeginterferon-α 2b) should be considered. In myelofibrosis, continuous risk assessment and symptom burden assessment are essential in guiding treatment selection. Allogeneic hematopoietic stem cell transplantation (allo-HSCT) in MF should always be based on accurate risk stratification for disease-risk and post-HSCT outcome. Management of classical Ph-negative MPN entails accurate diagnosis, cytogenetic and molecular evaluation, risk stratification, and treatment strategies that are outcome-oriented (curative, disease modification, improvement of quality-of-life).

* Title and MeSH Headings from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.