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Management of classical Philadelphia chromosome-negative myeloproliferative neoplasms in Asia: consensus of the Asian Myeloid Working Group

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Abstract

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).

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Authors and Affiliations

  1. Department of Medicine, LKS Faculty of Medicine, School of Clinical Medicine, The University of Hong Kong, Pok Fu Lam, Hong Kong, China

    Harinder Gill, Garret M. K. Leung & Yok-Lam Kwong

  2. Department of Hematology-Oncology, National University Cancer Institute, Singapore, Singapore

    Melissa G. M. Ooi, Winnie Z. Y. Teo & Wee-Joo Chng

  3. Department of Medicine, Yong Loo Lin School of Medicine, National University, Singapore, Singapore

    Melissa G. M. Ooi & Wee-Joo Chng

  4. Fast and Chronic Program, Alexandra Hospital, Singapore, Singapore

    Winnie Z. Y. Teo

  5. Department of Medicine, Sunway Medical Centre, Shah Alam, Selangor, Malaysia

    Chieh-Lee Wong

  6. Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea

    Chul Won Choi

  7. Department of Haematology, Singapore General Hospital, Singapore, Singapore

    Gee-Chuan Wong & Zhentang Lao

  8. King Chulalongkorn Memorial Hospital, Chulalongkorn University, Bangkok, Thailand

    Ponlapat Rojnuckarin

  9. University of Santo Tomas Hospital PH, Manila, Philippines

    Ma. Rosario Irene D. Castillo

  10. Blood Disease Hospital and Institute of Hematology, Chinese Academy of Medical Sciences Peking Union Medical College, Tianjin, China

    Zhijian Xiao

  11. Department of Internal Medicine, National Taiwan University Hospital, Taipei, Taiwan

    Hsin-An Hou

  12. Chang Gung Memorial Hospital-Linkou, Chang Gung University, Taoyuan, Taiwan

    Ming-Chung Kuo & Lee-Yung Shih

  13. University of Malaya, Kuala Lumpur, Malaysia

    Gin-Gin Gan

  14. Department of Laboratory Medicine, National Taiwan University Hospital, Taipei, Taiwan

    Chien-Chin Lin

  15. Department of Medicine, Professorial Block, Queen Mary Hospital, Pokfulam Road, Pok Fu Lam, Hong Kong, China

    Harinder Gill

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  1. Harinder Gill
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Contributions

HG and YLK conceived the idea of the consensus, wrote and approved the manuscript. GMKL, MGMO, WT, CLW, CWC, GCW, ZL, PR, IR, ZX, HAH, MCK, LYS, GGG, CCL and WJC reviewed and approved the manuscript.

Corresponding author

Correspondence to Harinder Gill.

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Conflict of interest

Harinder Gill has the following disclosures: research funding/grants from Celgene, Novartis Oncology and PharmaEssentia Corporation; investigator-initiated study collaborations with Imago Biosciences Inc., PharmaEssentia Corporation and Novartis; Consultancies for Abbvie, Astellas, BMS, GSK, Novartis Oncology and PharmaEssentia Corporation; Conference support from Novartis and PharmaEssentia Corporation. All other authors declare that they have no competing interest.

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Gill, H., Leung, G.M.K., Ooi, M.G.M. et al. Management of classical Philadelphia chromosome-negative myeloproliferative neoplasms in Asia: consensus of the Asian Myeloid Working Group. Clin Exp Med 23, 4199–4217 (2023). https://doi.org/10.1007/s10238-023-01189-9

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