Berti E, Tomasini D, Vermeer M H, Meijer C J, Alessi E, Willemze R
Institute of Dermatologic Science, IRCCS Ospedale Maggiore, Milan, Italy.
Am J Pathol. 1999 Aug;155(2):483-92. doi: 10.1016/S0002-9440(10)65144-9.
Cutaneous T cell lymphomas (CTCL) generally have the phenotype of CD3+, CD4+, CD45RO+ memory T cells. CTCL expressing a CD8+ T cell phenotype are extremely rare and ill-defined. To elucidate whether these CD8+ CTCL represent a distinct disease entity, the clinical, histological, and immunophenotypical features of 17 CD8+ CTCL were reviewed. None of the 17 cases expressed markers characteristic of natural killer cells or gamma/delta T cells. Nine of 17 cases showed the characteristic clinical and histological features as well as clinical behavior of well defined types of CTCL, such as mycosis fungoides (2 cases), pagetoid reticulosis (2 cases), lymphomatoid papulosis (2 cases), and CD30+ large T cell lymphoma (2 cases), all of which usually express a CD4+ T cell phenotype, and 1 case of subcutaneous panniculitis-like T cell lymphoma. The other 8 cases formed a homogeneous group showing a distinctive set of clinicopathological and immunophenotypical features, not consistent with that of other well defined types of CTCL. Clinical characteristics included presentation with generalized patches, plaques, papulonodules, and tumors mimicking disseminated pagetoid reticulosis; metastatic spread to unusual sites, such as the lung, testis, central nervous system, and oral cavity, but not to the lymph nodes; and an aggressive course (median survival, 32 months). Histologically, these lymphomas were characterized by band-like infiltrates consisting of pleomorphic T cells or immunoblasts, showing a diffuse infiltration of an acanthotic epidermis with variable degrees of spongiosis, intraepidermal blistering, and necrosis. The neoplastic cells showed a high Ki-67 proliferation index and expression of CD3, CD8, CD7, CD45RA, betaF1, and TIA-1 markers, whereas CD2 and CD5 were frequently lost. Expression of TIA-1 pointed out that these lymphomas are derived from a cytotoxic T cell subset. The results of this and other studies reviewed herein suggest that these strongly epidermotropic primary cutaneous CD8+ cytotoxic T cell lymphomas represent a distinct type of CTCL with an aggressive clinical behavior.
皮肤T细胞淋巴瘤（CTCL）通常具有CD3 +、CD4 +、CD45RO +记忆T细胞的表型。表达CD8 + T细胞表型的CTCL极为罕见且定义不明确。为了阐明这些CD8 + CTCL是否代表一种独特的疾病实体，我们回顾了17例CD8 + CTCL的临床、组织学和免疫表型特征。这17例病例均未表达自然杀伤细胞或γ/δ T细胞的特征性标志物。17例中有9例表现出明确类型的CTCL的特征性临床、组织学特征及临床行为，如蕈样肉芽肿（2例）、派杰样网状细胞增生症（2例）、淋巴瘤样丘疹病（2例）和CD30 +大T细胞淋巴瘤（2例），所有这些通常表达CD4 + T细胞表型，还有1例皮下脂膜炎样T细胞淋巴瘤。另外8例形成一个同质组，表现出一组独特的临床病理和免疫表型特征，与其他明确类型的CTCL不一致。临床特征包括表现为泛发性斑片、斑块、丘疹结节和肿瘤，类似播散性派杰样网状细胞增生症；转移至不常见部位，如肺、睾丸、中枢神经系统和口腔，但不累及淋巴结；病程侵袭性（中位生存期32个月）。组织学上，这些淋巴瘤的特征是由多形性T细胞或免疫母细胞组成的带状浸润，显示棘层肥厚的表皮弥漫性浸润，伴有不同程度的海绵形成、表皮内水疱形成和坏死。肿瘤细胞显示高Ki-67增殖指数以及CD3、CD8、CD7、CD45RA、βF1和TIA-1标志物的表达，而CD2和CD5常缺失。TIA-1的表达表明这些淋巴瘤源自细胞毒性T细胞亚群。本文回顾的这项研究及其他研究结果表明，这些具有强烈亲表皮性的原发性皮肤CD8 +细胞毒性T细胞淋巴瘤代表一种具有侵袭性临床行为的独特类型的CTCL。
