CNIO Lymphoma Group, Fundación Jiménez Díaz, Madrid, Spain.
Am J Surg Pathol. 2013 Mar;37(3):375-84. doi: 10.1097/PAS.0b013e318275d1a2.
Primary cutaneous γδ T-cell lymphomas (PCGD-TCLs) are considered a subgroup of aggressive cytotoxic T-cell lymphomas (CTCLs). We have taken advantage of a new, commercially available antibody that recognizes the T-cell receptor-γ (TCR-γ) subunit of the TCR in paraffin-embedded tissue. We have analyzed a series of 146 primary cutaneous T-cell lymphomas received for consultation or a second opinion in the CNIO Pathology Department. Cases were classified according to the World Health Organization 2008 classification as mycosis fungoides (MF; n=96), PCGD-TCLs (n=5), pagetoid reticulosis (n=6), CD30(+) primary cutaneous anaplastic large cell lymphomas (n=5), primary cutaneous CD8 aggressive epidermotropic CTCLs (n=3), primary cutaneous CTCL, not otherwise specified (n=4), and extranodal nasal-type NK/T-cell lymphomas primarily affecting the skin or subcutaneous tissue (n=11). Sixteen cases of the newly named lymphomatoid papulosis type D (LyP-D; n=16) were also included. In those cases positive for TCR-γ, a further panel of 13 antibodies was used for analysis, including TIA-1, granzyme B, and perforin. Clinical and follow-up data were recorded in all cases. Twelve cases (8.2%) were positive for TCR-γ, including 5 PCGD-TCLs, 2 MFs, and 5 LyP-Ds. All 5 PCGD-TCL patients and 1 MF patient died of the disease, whereas the other MF patient and all those with LyP-D were alive. All cases expressed cytotoxic markers, were frequently CD3(+)/CD8(+), and tended to lose CD5 and CD7 expressions. Eight of 12 and 5 of 11 cases were CD30(+) and CD56(+), respectively. Interestingly, 5/12 TCR-γ-positive cases also expressed TCR-BF1. All cases analyzed were negative for Epstein-Barr virus-encoded RNA. In conclusion, TCR-γ expression seems to be rare and is confined to cytotoxic primary cutaneous TCLs. Nevertheless, its expression is not exclusive to PCGD-TCLs, as TCR-γ protein can be found in other CTCLs. Moreover, its expression does not seem to be associated with bad prognosis by itself, as it can be found in cases with good and bad outcomes.
原发性皮肤 γδ T 细胞淋巴瘤(PCGD-TCL)被认为是侵袭性细胞毒性 T 细胞淋巴瘤(CTCL)的一个亚群。我们利用了一种新的、商业上可获得的抗体,该抗体可识别石蜡包埋组织中的 T 细胞受体-γ(TCR-γ)亚单位。我们分析了在 CNIO 病理科会诊或二次诊断的 146 例原发性皮肤 T 细胞淋巴瘤病例。根据 2008 年世界卫生组织分类,病例被分为蕈样真菌病(MF;n=96)、PCGD-TCL(n=5)、疱疹样网状细胞增生症(n=6)、CD30(+)原发性皮肤间变性大细胞淋巴瘤(n=5)、原发性皮肤 CD8 侵袭性表皮 T 细胞淋巴瘤(n=3)、未特指的原发性皮肤 CTCL(n=4)和结外鼻型 NK/T 细胞淋巴瘤主要累及皮肤或皮下组织(n=11)。还包括 16 例新命名的淋巴瘤样丘疹病 D 型(LyP-D;n=16)。在那些 TCR-γ 阳性的病例中,进一步使用了包括 TIA-1、颗粒酶 B 和穿孔素在内的 13 种抗体进行分析。在所有病例中都记录了临床和随访数据。12 例(8.2%)TCR-γ 阳性,包括 5 例 PCGD-TCL、2 例 MF 和 5 例 LyP-D。所有 5 例 PCGD-TCL 患者和 1 例 MF 患者均因疾病死亡,而另 1 例 MF 患者和所有 LyP-D 患者均存活。所有病例均表达细胞毒性标志物,通常为 CD3(+)/CD8(+),并倾向于失去 CD5 和 CD7 的表达。在 12 例中有 8 例 CD30(+)和 11 例中有 5 例 CD56(+)。有趣的是,在 5/12 TCR-γ 阳性病例中也表达 TCR-BF1。所有分析的病例均为 EBV 编码的 RNA 阴性。总之,TCR-γ 的表达似乎很少见,仅限于细胞毒性原发性皮肤 TCL。然而,它的表达并不局限于 PCGD-TCL,因为 TCR-γ 蛋白也可以在其他 CTCL 中找到。此外,其表达本身似乎与不良预后无关,因为它可以在预后良好和不良的病例中发现。