中华皮肤科杂志 ›› 2026, e20240605.doi: 10.35541/cjd.20240605

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[开放获取]    第5版WHO造血与淋巴组织肿瘤分类(WHO-HAEM5)皮肤淋巴瘤分类更新解读

庞智屿    刘洁   

  1. 中国医学科学院  北京协和医学院  北京协和医院皮肤科,疑难重症及罕见病国家重点实验室,北京  100730
  • 收稿日期:2024-11-07 修回日期:2026-01-02 发布日期:2026-04-27
  • 通讯作者: 刘洁 E-mail:Liujie04672@pumch.cn
  • 基金资助:
    中国医学科学院医学与健康科技创新工程项目(2025-I2M-C&T-A-002);朝阳区数字医疗概念验证计划项目(2025SLZD007);国家临床重点专科建设项目

Interpretation of updates in the classification of cutaneous lymphomas in the 5th edition of the WHO Classification of Haematolymphoid Tumours (WHO-HAEM5)

Pang Zhiyu, Liu Jie    

  1. Department of Dermatology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China
  • Received:2024-11-07 Revised:2026-01-02 Published:2026-04-27
  • Contact: Liu Jie E-mail:Liujie04672@pumch.cn
  • Supported by:
    CAMS Innovation Fund for Medical Sciences (2025-I2M-C&T-A-002); Beijing Chaoyang Digital Health Proof of Concept Project (2025SLZD007); National Key Clinical Specialty Project of China

摘要: 【摘要】 原发性皮肤淋巴瘤(PCL)是一组具有独特临床表现和组织学特征的异质性疾病,诊断和治疗存在一定挑战。2022年发布的第5版WHO造血与淋巴组织肿瘤分类(WHO-HAEM5)对PCL的分类进行了更新,涉及皮肤T细胞淋巴瘤(CTCL)和皮肤B细胞淋巴瘤的多种亚型。本次更新细化了对少见CTCL亚型的定义和归类,并将Sézary综合征从CTCL中移出,重新归类为成熟T细胞和自然杀伤细胞白血病。此外,PCL的分子遗传学研究取得新进展,反映在不同亚型的具体分类中。本文结合WHO-HAEM5的主要变化,对PCL分类更新内容进行解读,为临床实践提供参考。

关键词: 淋巴瘤, 皮肤淋巴瘤, 淋巴瘤, T细胞, 皮肤, 淋巴瘤, B细胞, WHO-HAEM5, 分类解读

Abstract: 【Abstract】 Primary cutaneous lymphomas (PCLs) are a heterogeneous group of diseases with distinct clinical manifestations and histopathological features, and their diagnosis and treatment remain challenging. The 5th edition of the WHO Classification of Haematolymphoid Tumours (WHO-HAEM5), published in 2022, introduced updates to the classification of PCLs, involving multiple subtypes of cutaneous T-cell lymphomas (CTCL) and cutaneous B-cell lymphomas. This update further refines the definition and classification of rare CTCL subtypes and reclassifies Sézary syndrome, removing it from CTCL and categorizing it as a mature T-cell and natural killer cell leukemia. In addition, advances in the molecular genetics of PCLs have been incorporated into the classification of specific subtypes. Based on the major changes in WHO-HAEM5, this article provides an interpretation of the updated classification of PCLs, aiming to provide a reference for clinical practice.

Key words: Lymphoma, Cutaneous lymphomas, Lymphoma, T-Cell, cutaneous, Lymphoma, B-Cell, WHO-HAEM5, Classification interpretation

引用本文

庞智屿 刘洁. [开放获取]    第5版WHO造血与淋巴组织肿瘤分类(WHO-HAEM5)皮肤淋巴瘤分类更新解读[J]. 中华皮肤科杂志, 2026,e20240605. doi:10.35541/cjd.20240605

Pang Zhiyu, Liu Jie . Interpretation of updates in the classification of cutaneous lymphomas in the 5th edition of the WHO Classification of Haematolymphoid Tumours (WHO-HAEM5)[J]. Chinese Journal of Dermatology,2026,e20240605. doi:10.35541/cjd.20240605