Department of Dermatology, AP-HP, Saint-Louis Hospital, Paris, France.
Dermatology. 2011;222(4):297-303. doi: 10.1159/000327932. Epub 2011 May 21.
Cutaneous γ/δ T-cell lymphoma (CGD-TCL) is a recent entity described in the newly revised World Health Organization-European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas, and is characterized by the γ/δ T-cell receptor expression on atypical lymphocytes. Only a few cases of primary CGD-TCL have been reported, with an extremely aggressive course (median survival time of 15 months). We describe 2 atypical cases of CGD-TCL. The first case was initially misdiagnosed as an inflammatory panniculitis due to the granulomatous infiltrate on the skin biopsy specimen. Diagnosis was confirmed using δ PCR that revealed γ/δ T-cell clonal expansion. The evolution was marked by predominant γ/δ T-cell infiltrate with diffuse body fat involvement as seen on positron emission tomography-computed tomography. The second case is the first described Epstein-Barr virus (EBV)-associated CGD-TCL with a rapidly fatal evolution. CGD-TCL is also a heterogeneous entity and δ PCR and EBV-encoded RNA probe to detect an EBV latent infection may help diagnose and characterize these cutaneous lymphomas.
皮肤 γ/δ T 细胞淋巴瘤（CGD-TCL）是新近在世界卫生组织-欧洲癌症研究与治疗组织修订的皮肤淋巴瘤分类中描述的一种实体，其特征在于不典型淋巴细胞上的 γ/δ T 细胞受体表达。仅有少数原发性 CGD-TCL 病例报道，其病程极具侵袭性（中位生存时间为 15 个月）。我们描述了 2 例不典型 CGD-TCL 病例。第 1 例最初被误诊为炎症性脂膜炎，因为皮肤活检标本上有肉芽肿浸润。使用 δ PCR 证实了 γ/δ T 细胞克隆性扩增，从而确诊。其进展以弥漫性全身脂肪受累为主，伴有 γ/δ T 细胞弥漫浸润，正电子发射断层扫描-计算机断层扫描可见。第 2 例是首例描述的 EBV 相关 CGD-TCL，其进展迅速且致命。CGD-TCL 也是一种异质性实体，δ PCR 和 EBV 编码 RNA 探针检测 EBV 潜伏感染可能有助于诊断和表征这些皮肤淋巴瘤。
