Tan S H, Sim C S, Ong B H
Institute of Dermatology Singapore, National Skin Centre, 1 Mandalay Road, Singapore 308205.
Br J Dermatol. 2003 Sep;149(3):542-53. doi: 10.1046/j.1365-2133.2003.05476.x.
Cutaneous lymphomas other than mycosis fungoides (MF) are a heterogeneous group with wide variations in clinical presentation, biological behaviour and prognosis. New classification systems have been designed or proposed in recent years, with well-defined disease entities and emphasis on the importance of site.
This study aims to analyse a series of non-MF lymphomas in an institution-based dermatological setting in Singapore, based on the European Organization for Research and Treatment of Cancer (EORTC) classification and the World Health Organization (WHO) classification. A secondary objective is to highlight the clinical utility of both classification systems.
Forty cases diagnosed over a 12-year period were examined by immunohistochemistry with antibodies targeting CD3, CD4, CD5, CD8, CD20, CD30, CD43, CD45RO, CD56 and CD68 in paraffin-embedded specimens. The immunohistological diagnosis was correlated with the clinical presentation and staging investigations for the final diagnosis and the course of disease recorded.
Non-MF T-cell lymphomas presenting in the skin comprised 31 cases (78%) and were 3(1/2) times more common than B-cell lymphomas, which comprised nine cases (22%). The common subtypes were lymphomatoid papulosis, CD30+ large cell cutaneous T-cell lymphoma and subcutaneous panniculitis-like T-cell lymphoma. The commonly ascribed B-cell pattern with infiltrates in the mid and deep dermis and perivascular spaces was seen in 60% of T-cell lymphomas. Overall, there were equal numbers of primary cutaneous T-cell lymphomas and those due to concurrent or secondary cutaneous lymphoma. Five of six cases of subcutaneous panniculitis-like T-cell lymphoma had concurrent cutaneous and systemic involvement and their median survival was 7 months.
The predominance of cutaneous T-cell lymphomas in this case series closely matched that reported from east Asia; cutaneous B-cell lymphomas are much less common than in Europe. The EORTC classification, which is designed only for primary cutaneous lymphomas, should be used in conjunction with the WHO classification because of the high prevalence of cutaneous lymphomas as the secondary site of disease from systemic lymphoma. In addition, subcutaneous panniculitis-like T-cell lymphoma is a primary cutaneous lymphoma where systemic involvement is common at initial presentation. We propose full immunophenotyping and complete clinical evaluation with staging investigations for all patients presenting with cutaneous lymphomas other than MF.
除蕈样肉芽肿（MF）外的皮肤淋巴瘤是一组异质性疾病，临床表现、生物学行为和预后差异很大。近年来已设计或提出了新的分类系统，疾病实体明确，并强调了部位的重要性。
本研究旨在基于欧洲癌症研究与治疗组织（EORTC）分类和世界卫生组织（WHO）分类，分析新加坡一家皮肤病机构的一系列非MF淋巴瘤。次要目的是突出这两种分类系统的临床实用性。
对12年间诊断的40例病例进行免疫组织化学检查，使用针对石蜡包埋标本中CD3、CD4、CD5、CD8、CD20、CD30、CD43、CD45RO、CD56和CD68的抗体。免疫组织学诊断与临床表现和分期检查相关，以做出最终诊断并记录疾病进程。
皮肤中出现的非MF T细胞淋巴瘤有31例（78%），比B细胞淋巴瘤常见3.5倍，B细胞淋巴瘤有9例（22%）。常见亚型为淋巴瘤样丘疹病、CD30+大细胞皮肤T细胞淋巴瘤和皮下脂膜炎样T细胞淋巴瘤。60%的T细胞淋巴瘤可见常见的B细胞模式，表现为真皮中层和深层以及血管周围间隙浸润。总体而言，原发性皮肤T细胞淋巴瘤与并发或继发性皮肤淋巴瘤的数量相等。六例皮下脂膜炎样T细胞淋巴瘤中有五例同时有皮肤和全身受累，它们的中位生存期为7个月。
本病例系列中皮肤T细胞淋巴瘤的优势与东亚报道的情况密切匹配；皮肤B细胞淋巴瘤比欧洲少见得多。EORTC分类仅针对原发性皮肤淋巴瘤设计，由于皮肤淋巴瘤作为系统性淋巴瘤的次要疾病部位患病率较高，应与WHO分类结合使用。此外，皮下脂膜炎样T细胞淋巴瘤是一种原发性皮肤淋巴瘤，初始表现时全身受累很常见。我们建议对所有出现非MF皮肤淋巴瘤的患者进行全面的免疫表型分析和完整的临床评估及分期检查。
