Dermatology Department, Andreas Sygros Hospital, University of Athens, Athens 17456, Greece.
Br J Dermatol. 2009 Oct;161(4):826-30. doi: 10.1111/j.1365-2133.2009.09301.x. Epub 2009 May 8.
Fewer than 5% of cases of mycosis fungoides (MF) present with a cytotoxic/suppressor CD8+ phenotype which, despite immunophenotypic similarities with CD8+ aggressive lymphomas, is regarded as a phenotypic variant of MF. Poikilodermatous MF showing a CD8+ phenotype has been reported to have a nonaggressive clinical behaviour and a good response to psoralen plus ultraviolet A treatment.
To perform a retrospective study of CD8+ MF cases diagnosed in the skin lymphoma clinic of Andreas Sygros Hospital.
We analysed the clinical characteristics, the immunophenotypic and molecular indices, as well as the clinical course of these patients.
Seven cases of CD8+ MF (6.5% of all cases of cutaneous T-cell lymphoma) were diagnosed during 2002-2007. One of seven patients had stage IA, five stage IB and one stage IIB disease. Clinical characteristics were variable: four of seven patients presented with poikilodermatous plaques (in one of them lesions of lymphomatoid papulosis with CD8+ phenotype coexisted), one patient with classic MF, one with plantar MF and one with follicular MF. The time period between disease onset and diagnosis was long for most patients (up to 33 years). All patients received the recommended treatment according to TNM staging. Five of seven patients had complete remission, one partial response and one stable disease.
Special clinical characteristics, such as hyperpigmentation and poikiloderma, are often noted in CD8+ MF cases. In our series CD8+ MF presented with a long-standing disease and indolent course suggesting that CD8+ cytotoxic immunophenotype may represent a marker of mild biological behaviour.
蕈样肉芽肿（MF）中少于 5%的病例表现出细胞毒性/抑制性 CD8+表型，尽管其免疫表型与 CD8+侵袭性淋巴瘤相似，但被认为是 MF 的一种表型变异。表现出 CD8+表型的斑驳状 MF 已被报道具有非侵袭性的临床行为和对补骨脂素加紫外线 A 治疗的良好反应。
对 Andreas Sygros 医院皮肤淋巴瘤诊所诊断的 CD8+MF 病例进行回顾性研究。
我们分析了这些患者的临床特征、免疫表型和分子指标以及临床病程。
2002-2007 年间诊断出 7 例 CD8+MF（所有皮肤 T 细胞淋巴瘤病例的 6.5%）。7 例患者中有 1 例为 IA 期，5 例为 IB 期，1 例为 IIB 期。临床特征多样：7 例患者中有 4 例表现为斑驳状斑块（其中 1 例存在 CD8+表型的蕈样肉芽肿样病变），1 例为经典 MF，1 例为跖部 MF，1 例为滤泡性 MF。大多数患者从发病到诊断的时间间隔较长（长达 33 年）。所有患者均根据 TNM 分期接受了推荐的治疗。7 例患者中有 5 例完全缓解，1 例部分缓解，1 例病情稳定。
CD8+MF 病例常出现特殊的临床特征，如色素沉着和斑驳。在我们的系列中，CD8+MF 表现为长期疾病和惰性病程，这表明 CD8+细胞毒性免疫表型可能是轻度生物学行为的标志物。
