Although the vast majority of CTCL subtypes are of the CD4+ T-helper cell differentiation phenotype, there is a spectrum of CD8+ variants that manifest wide-ranging clinical, histologic, and phenotypic features that inform the classification of the disease. CD8, like CD4, and cytotoxic molecules (including TIA and granzyme) are readily detectable via IHC staining of tissue and, when expressed on the phenotypically abnormal T-cell population, can help distinguish specific CTCL subtypes. Nonetheless, given that the histopathologic differential for CD8+ lymphoproliferative disorders and lymphomas may range from very indolent lymphomatoid papulosis (LyP) to aggressive entities like CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma (AECTCL), CD8 and/or cytotoxic molecule expression alone is insufficient for diagnosis and is not in itself an indicator of prognosis. We present a review of CTCL subtypes that can demonstrate CD8 positivity: CD8+ mycosis fungoides (MF), LyP type D, subcutaneous panniculitis-like T-cell lymphoma (SPTCL), primary cutaneous gamma/delta T-cell lymphoma (PCGDTL), CD8+ AECTCL, and acral CD8+ T-cell lymphoproliferative disorder (acral CD8+ TCLPD). These diseases may have different clinical manifestations and distinctive treatment algorithms. Due to the rare nature of these diseases, it is imperative to integrate clinical, histologic, and immunohistochemical findings to determine an accurate diagnosis and an appropriate treatment plan.
尽管绝大多数皮肤T细胞淋巴瘤(CTCL)亚型表现为CD4+辅助性T细胞分化表型,但存在一系列CD8+变异型,其临床表现、组织学特征及表型特点差异显著,这些差异为疾病分类提供了依据。与CD4类似,CD8及细胞毒性分子(包括TIA和颗粒酶)可通过组织免疫组化染色检测,当这些标记物在表型异常的T细胞群中表达时,有助于区分特定的CTCL亚型。然而,鉴于CD8+淋巴增殖性疾病和淋巴瘤的组织病理学鉴别范围广泛,可从惰性的淋巴瘤样丘疹病(LyP)D型到侵袭性实体如CD8+侵袭性亲表皮性细胞毒性T细胞淋巴瘤(AECTCL),仅凭CD8和/或细胞毒性分子的表达不足以确诊,其本身亦不能作为预后指标。本文综述了可表现为CD8阳性的CTCL亚型:CD8+蕈样肉芽肿(MF)、LyP D型、皮下脂膜炎样T细胞淋巴瘤(SPTCL)、原发性皮肤γ/δ T细胞淋巴瘤(PCGDTL)、CD8+ AECTCL以及肢端CD8+ T细胞淋巴增殖性疾病(肢端CD8+ TCLPD)。这些疾病可能具有不同的临床表现和独特的治疗方案。由于这些疾病较为罕见,必须综合临床、组织学和免疫组化结果,以确定准确的诊断并制定合适的治疗计划。
Clinical and Histologic Variants of CD8+ Cutaneous T-Cell Lymphomas