Department of Pediatrics, Division Hematology-Oncology, Emory School of Medicine, The Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta, Atlanta, GA, USA.
Department of Pediatrics, Tufts University School of Medicine, Institute for Clinical Research and Health Policy Studies, Tufts Medical Center, Boston, MA, USA.
Br J Haematol. 2019 Dec;187(5):573-587. doi: 10.1111/bjh.16197. Epub 2019 Sep 30.
The treatment of Hodgkin lymphoma (HL) is one of early success. However, disease-free survival (DFS) does not reflect latent organ injury and its impact on health status and well-being beyond 5 years. In fact, we are at a crossroads, in terms of needing individualized approaches to maintain DFS, while minimizing late effects and preserving health-related quality of life (HRQoL). Premature morbidity and mortality translate to a high societal cost associated with the potential number of productive life years ahead in this population who are young at diagnosis. The discordance between short-term lymphoma-free survival and long-term health and HRQoL creates a "survivorship gap" which can be characterized for individuals and for subgroups of patients. The current review delineates contributors to compromised outcomes and health status in child and adolescent (paediatric) HL and frames the survivorship gap in terms of primary and secondary prevention. Primary prevention aims to titrate therapy. Secondary prevention entails strategies to intervene against late effects. Bridging the survivorship gap will be attained with enhanced knowledge of and attention to biology of the tumour and microenvironment, host genetic factors, HRQoL and sub-populations with disparate outcomes.
霍奇金淋巴瘤(HL)的治疗是早期成功的典范之一。然而,无病生存(DFS)并不能反映潜在的器官损伤及其对 5 年以上健康状况和幸福感的影响。事实上,我们正处于一个十字路口,需要个体化的方法来维持 DFS,同时最大限度地减少晚期效应并保持与健康相关的生活质量(HRQoL)。发病时年轻的 HL 患者,因过早发病和死亡导致的高社会成本,与其预期的未来有生产力的生活年限相关。短期淋巴瘤无生存和长期健康及 HRQoL 之间的不匹配导致了“生存差距”,这可以从个体和患者亚组的角度进行描述。目前的综述阐述了导致儿童和青少年(儿科)HL 不良结局和健康状况的因素,并从一级和二级预防的角度来阐述生存差距。一级预防旨在调整治疗。二级预防需要针对晚期效应的干预策略。通过提高对肿瘤和微环境生物学、宿主遗传因素、HRQoL 以及具有不同结局的亚人群的认识和关注,将实现缩小生存差距的目标。
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