Kempf Werner, Kazakov Dmitry V, Kerl Katrin
*Dermatopathologist and Dermatologist, Kempf und Pfaltz, Histologische Diagnostik, Zürich, Switzerland; †Professor, Department of Dermatology, University Hospital Zürich, Switzerland; ‡Dermatopathologist and Dermatologist, Professor, Department of Pathology, Medical Faculty in Pilsen, Charles University, Prague, Czech Republic; and §Dermatopathologist and Dermatologist, Faculty Member, Department of Dermatology, University Hospital Zürich, Switzerland.
Am J Dermatopathol. 2014 Feb;36(2):105-23. doi: 10.1097/DAD.0b013e318289b1db.
Primary cutaneous T-cell lymphomas (CTCL) represent the majority of cutaneous lymphomas (CLs) and are a spectrum of diseases with a wide variety of clinical, histological, and phenotypic features and diverse biologic behavior. This review focuses on the observations on new variants of CTCL and recent developments in deciphering the pathogenetic mechanisms, which have implication for the nosologic concepts and future classification of CLs. Variants of mycosis fungoides (MF) such as the unilesional and the papular form have been characterized in more detail. Studies analyzed the expression of CD30 and PD-1 in MF and other forms of CTCL. New variants in the group of cutaneous CD30⁺ lymphoproliferative disorders include the epidermotropic CD8⁺ variant of lymphomatoid papulosis (type D) and angiocentric lymphomatoid papulosis (type E), which histologically mimic aggressive lymphomas, and therefore may be diagnostically challenging. Cutaneous proliferations of T cell-expressing markers of follicular helper T cells (PD-1, CXCL-13, and bcl-6) display a prognostically heterogeneous group. There is an increasing spectrum of CTCL with the expression of CD8 by tumor cells, including CD8⁺ MF, CD8⁺ forms of cutaneous CD30⁺ lymphoproliferative disorders, and CD8⁺ small/medium-sized lymphoproliferations, which are not included as distinct entities in the World Health Organization-European Organization for Research and Treatment of Cancer for CLs and the World Health Organization classification. Unusual presentations and incomplete phenotypes of blastic neoplasm of plasmacytoid dendritic cells are discussed. Clinicopathologic correlation is mandatory for the diagnosis of primary CLs. Analysis of genetic and epigenetic alterations in CLs revealed new diagnostic markers and putative targets for therapy of aggressive forms of CLs.
原发性皮肤T细胞淋巴瘤（CTCL）是皮肤淋巴瘤（CL）的主要类型，是一系列具有广泛临床、组织学和表型特征以及多样生物学行为的疾病。本综述聚焦于CTCL新变种的观察结果以及在解析发病机制方面的最新进展，这些对CL的疾病分类概念和未来分类具有重要意义。蕈样肉芽肿（MF）的变种，如单病灶型和丘疹型，已得到更详细的描述。研究分析了MF及其他形式CTCL中CD30和PD-1的表达。皮肤CD30⁺淋巴增殖性疾病组中的新变种包括淋巴瘤样丘疹病的亲表皮性CD8⁺变种（D型）和血管中心性淋巴瘤样丘疹病（E型），其组织学表现类似侵袭性淋巴瘤，因此在诊断上可能具有挑战性。表达滤泡辅助性T细胞标志物（PD-1、CXCL-13和bcl-6）的T细胞皮肤增殖性病变显示出预后异质性群体。肿瘤细胞表达CD8的CTCL谱系不断增加，包括CD8⁺MF、皮肤CD30⁺淋巴增殖性疾病的CD8⁺形式以及CD8⁺小/中型淋巴增殖性病变，这些在世界卫生组织-欧洲癌症研究与治疗组织的CL分类和世界卫生组织分类中并未作为独立实体纳入。本文还讨论了浆细胞样树突状细胞母细胞性肿瘤的不寻常表现和不完全表型。原发性CL的诊断必须进行临床病理相关性分析。对CL的基因和表观遗传改变分析揭示了新的诊断标志物以及侵袭性CL治疗的潜在靶点。
