https://scholars.lib.ntu.edu.tw/handle/123456789/637177
標題: | Management of classical Philadelphia chromosome-negative myeloproliferative neoplasms in Asia: consensus of the Asian Myeloid Working Group | 作者: | Gill, Harinder Leung, Garret M K Ooi, Melissa G M Teo, Winnie Z Y Wong, Chieh-Lee Choi, Chul Won Wong, Gee-Chuan Lao, Zhentang Rojnuckarin, Ponlapat Castillo, Ma Rosario Irene D Xiao, Zhijian HSIN-AN HOU Kuo, Ming-Chung Shih, Lee-Yung Gan, Gin-Gin CHIEN-CHIN LIN Chng, Wee-Joo Kwong, Yok-Lam |
關鍵字: | Asia; Consensus; Guidelines; Myeloproliferative neoplasm; Treatment | 公開日期: | 25-九月-2023 | 來源出版物: | Clinical and experimental medicine | 摘要: | 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). |
URI: | https://scholars.lib.ntu.edu.tw/handle/123456789/637177 | ISSN: | 15918890 | DOI: | 10.1007/s10238-023-01189-9 |
顯示於: | 醫學院附設醫院 (臺大醫院) |
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