Kempf Werner
Kempf und Pfaltz, Histologische Diagnostik, Zürich, Switzerland; Department of Dermatology, University Hospital Zurich, CH-8091, Zurich, Switzerland.
Semin Diagn Pathol. 2017 Jan;34(1):22-35. doi: 10.1053/j.semdp.2016.11.005. Epub 2016 Nov 29.
Cutaneous CD30+ T-cell lymphoproliferative disorders (CD30+ T-LPD) represent a spectrum encompassing lymphomatoid papulosis (LyP), primary cutaneous anaplastic large-cell lymphoma (pcALCL) and borderline lesions. They share the expression of CD30 as a common phenotypic marker. They differ however in their clinical presentation, the histological features and clinical course. Moreover, LyP and PcALCL show numerous clinical, histological and phenotypic variants. Overlapping features of LyP and pcALCL with themselves and with other cutaneous and systemic lymphomas emphasize the importance of careful clinicopathologic correlation and staging in the diagnosis of CD30+ T-LPD. Furthermore, an increasing number of inflammatory and infectious skin disorders harboring medium-sized to large CD30+ cells have to be considered in the differential diagnosis. Whereas the expression of CD30 in cutaneous CD30+ T-LPD stands for a favourable prognosis, its expression in other cutaneous and systemic lymphomas has a divergent impact. The assessment of CD30 expression does not only provide prognostic information, but is of potential therapeutic relevance as CD30 can serve as a therapeutic target. This review focuses on the clinicopathological and phenotypic spectrum of CD30+ T-LPD, its differential diagnoses and the role of CD30 as a diagnostic, prognostic and therapeutic marker.
皮肤CD30+ T细胞淋巴增殖性疾病(CD30+ T-LPD)是一个谱系,包括淋巴瘤样丘疹病(LyP)、原发性皮肤间变性大细胞淋巴瘤(pcALCL)和交界性病变。它们都表达CD30这一共同的表型标志物。然而,它们在临床表现、组织学特征和临床病程方面存在差异。此外,LyP和PcALCL有许多临床、组织学和表型变异。LyP和pcALCL自身之间以及与其他皮肤和系统性淋巴瘤的重叠特征强调了在CD30+ T-LPD诊断中仔细进行临床病理相关性分析和分期的重要性。此外,在鉴别诊断中必须考虑越来越多含有中等大小至大的CD30+细胞的炎症性和感染性皮肤疾病。虽然CD30在皮肤CD30+ T-LPD中的表达代表预后良好,但其在其他皮肤和系统性淋巴瘤中的表达影响不一。评估CD30表达不仅能提供预后信息,而且由于CD30可作为治疗靶点,因此具有潜在的治疗相关性。本综述重点关注CD30+ T-LPD的临床病理和表型谱系、其鉴别诊断以及CD30作为诊断、预后和治疗标志物的作用。