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皮肤 T 细胞淋巴瘤:增强临床实践的实用建议。

Cutaneous T-cell lymphoma: practical recommendations to enhance clinical practice.

机构信息

Skin Lymphoma Group, University Hospital Birmingham, Birmingham, UK.

出版信息

Br J Hosp Med (Lond). 2021 Mar 2;82(3):1-6. doi: 10.12968/hmed.2021.0055. Epub 2021 Mar 13.

DOI:10.12968/hmed.2021.0055
PMID:33792384
Abstract

Management of cutaneous T-cell lymphoma should provide a holistic approach to a patient's wellbeing. Treatments depend on the stage of lymphoma. Patients with the early stages tend to have a near-normal life expectancy. Management should be aimed at improving the extent of disease and reducing symptoms with minimal therapeutic adverse effects. Skin-directed treatments are preferred and may be used in combination with treatments for symptom relief such as anti-pruritic medication. In advanced stages of disease where the median life expectancy is reduced the aims are also to prevent disease progression and prolong life, and this requires a multidisciplinary approach. Symptom control remains important as patients often have painful, itchy disfiguring lesions which greatly impact on health-related quality of life. National and international guidelines provide stage-related treatment options to be considered with first-line options followed by subsequent second-line therapies. All are listed in no particular order of preference and are chosen according to patients' needs and expertise of the treating centre. Several first-line options may be chosen before moving to the second-line options. Three drugs received European Medicines Agency approval in 2017 and 2018 (chlormethine gel, brentuximab and mogamulizumab) but there still remains an unmet need for more improved treatments or combinations. Most treatments only result in a partial response and there is no cure for early-stage disease; as such, patients live for a long time with their disease. In the advanced stages if a good response is achieved eligible patients will be considered for an allogeneic haematopoietic stem cell transplant.

摘要

皮肤 T 细胞淋巴瘤的治疗应采用整体方法来提高患者的生活质量。治疗方法取决于淋巴瘤的分期。早期患者的预期寿命接近正常。治疗的目的应是改善疾病的严重程度并减少治疗的不良反应,同时缓解症状。皮肤靶向治疗是首选方法,可与缓解症状的治疗方法联合使用,如止痒药物。在疾病的晚期,中位预期寿命缩短,治疗的目的也是预防疾病进展和延长生命,这需要多学科的治疗方法。症状控制仍然很重要,因为患者经常有疼痛、瘙痒、毁容的病变,这极大地影响了他们的生活质量。国家和国际指南提供了与分期相关的治疗选择,一线治疗方案之后是二线治疗方案。所有方案均未按偏好顺序列出,而是根据患者的需求和治疗中心的专业知识选择。在使用二线方案之前,可能会选择几种一线方案。2017 年和 2018 年,有三种药物获得了欧洲药品管理局的批准(氯苯丁嗪凝胶、 Brentuximab 和 Mogamulizumab),但仍需要更多改进的治疗方法或联合治疗方案。大多数治疗方法仅能获得部分缓解,早期疾病无法治愈;因此,患者长期带病生存。在疾病晚期,如果获得良好的缓解效果,有资格的患者将被考虑进行异基因造血干细胞移植。

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