我的管理方法:鞘膜细胞淋巴瘤的温和与侵略性疾病。
How I manage mantle cell lymphoma: indolent versus aggressive disease.
发表日期:2023 Feb 20
作者:
Matthew R Wilson, Aisling Barrett, Chan Yoon Cheah, Toby A Eyre
来源:
BRITISH JOURNAL OF HAEMATOLOGY
摘要:
Mantle cell lymphoma (MCL)是一种成熟的B细胞淋巴瘤,具有不同的临床进展和历史上不良的预后。由于病程的异质性(现在已经认识到有渐进型和侵袭性亚型),处理具有挑战性。渐进型MCL通常以白血病表现、SOX11阴性和低增殖指数(Ki-67)为特征。侵袭性MCL是以迅速扩散的淋巴结肿大、额外淋巴结受累、类母细胞或多形性组织学和高Ki-67为特征。侵袭性MCL中的肿瘤蛋白p53(TP53)异常是已认识的,对生存率带来明显负面影响。直到最近,试验并没有分别针对这些特定的亚型进行研究。随着有针对性的新型药物和细胞疗法的日益普及,治疗格局不断发展。在本综述中,我们描述了渐进型和侵袭性MCL的临床表现、生物因素和特定管理注意事项,并讨论当前和潜在的未来证据,这些证据可能有助于实现更个性化的治疗方法。©2023年英国血液学会和John Wiley & Sons Ltd。
Mantle cell lymphoma (MCL) is a mature B-cell lymphoma with a variable clinical course and historically poor prognosis. Management is challenging in part due to the heterogeneity of the disease course, with indolent and aggressive subtypes now well recognised. Indolent MCL is often characterised by a leukaemic presentation, SOX11 negativity and low proliferation index (Ki-67). Aggressive MCL is characterised by rapid onset widespread lymphadenopathy, extra-nodal involvement, blastoid or pleomorphic histology and high Ki-67. Tumour protein p53 (TP53) aberrations in aggressive MCL are recognised with clear negative impact on survival. Until recently, trials have not addressed these specific subtypes separately. With the increasing availability of targeted novel agents and cellular therapies, the treatment landscape is constantly evolving. In this review, we describe the clinical presentation, biological factors, and specific management considerations of both indolent and aggressive MCL and discuss current and potential future evidence which may help move to a more personalised approach.© 2023 British Society for Haematology and John Wiley & Sons Ltd.