Kumar S, Krenacs L, Medeiros J, Elenitoba-Johnson K S, Greiner T C, Sorbara L, Kingma D W, Raffeld M, Jaffe E S
Hematopathology Section, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892-1500, USA.
Hum Pathol. 1998 Apr;29(4):397-403. doi: 10.1016/s0046-8177(98)90122-8.
Subcutaneous panniculitic T cell lymphoma (SCPTCL) is characterized by primary involvement of the subcutaneous fat in a manner mimicking panniculitis. We studied 16 cases of this lymphoma to define its immunophenotypical profile as well as cellular origin. Involvement of the subcutaneous fat in a lacelike pattern with neoplastic cells rimming individual fat spaces was present in all cases. All 16 cases were of T cell phenotype. Thirteen of the 16 cases were CD8+, whereas three were negative for both CD4 and CD8. Twelve cases were stained for betaF1; of these, eight were betaF1+ and four were betaF1-. Focal staining for CD56 and CD30 was seen in 2 of 13 and two of eight cases, respectively. Intense diffuse positivity for the cytotoxic granular proteins T cell intracellular antigen-1 (TIA-1) and perforin was present in all cases, indicating an origin from cytotoxic T lymphocytes. Ten cases studied for Epstein-Barr viral sequences were negative. Eight of 9 cases with amplifiable DNA showed a clonal TCR gamma gene rearrangement by polymerase chain reaction. Controls included seven cases of benign panniculitis and seven other peripheral T cell lymphomas involving the skin and subcutaneous tissues: two peripheral T cell lymphomas, not otherwise specified (PTL,NOS), four anaplastic large cell lymphomas (ALCL), one T/NK cell lymphoma. The seven cases of panniculitis lacked cytological atypia and were characterized by an admixture of CD4+ and CD8+ cells with interspersed aggregates of L26+ B cells. Only infrequent cells showed staining for TIA-1 and perforin. In the control cases of T cell lymphoma, the infiltrate had a tendency for dermal and sometimes even epidermal involvement, with sheeting out of malignant cells, in contrast to the characteristic subcutaneous localization and rimming of fat spaces noted in SCPTCL. The two PTL, NOS were CD4+ and negative for both TIA-1 and perforin. Although the remaining controls expressed TIA-1 and perforin, in keeping with their cytotoxic T or natural killer (NK) cell origin, histological and other immunophenotypical features allowed distinction from SCPTCL. Five cases of SCPTCL were also stained for apoptosis using a tdt-mediated end labeling kit. All cases showed numerous positive apoptotic bodies, suggesting apoptosis as the mechanism of cell death in these tumors. Our study indicates that SCPTCL constitutes a distinctive clinicopathological entity derived from cytotoxic T lymphocytes and should be differentiated from other benign and malignant lymphoid infiltrates involving the subcutis. The apoptosis seen in these tumors may be mediated by release of cytotoxic granular proteins.
皮下脂膜炎样T细胞淋巴瘤(SCPTCL)的特征是皮下脂肪原发性受累,其方式类似脂膜炎。我们研究了16例这种淋巴瘤,以确定其免疫表型特征以及细胞起源。所有病例均可见皮下脂肪呈花边状受累,肿瘤细胞围绕单个脂肪间隙。所有16例均为T细胞表型。16例中有13例CD8阳性,而3例CD4和CD8均为阴性。12例进行了βF1染色;其中8例βF1阳性,4例βF1阴性。13例中有2例、8例中有2例分别可见CD56和CD30的局灶性染色。所有病例中细胞毒性颗粒蛋白T细胞细胞内抗原-1(TIA-1)和穿孔素均呈强烈弥漫性阳性,表明其起源于细胞毒性T淋巴细胞。对10例进行爱泼斯坦-巴尔病毒序列检测均为阴性。9例可扩增DNA的病例中有8例通过聚合酶链反应显示克隆性TCRγ基因重排。对照包括7例良性脂膜炎和7例其他累及皮肤和皮下组织的外周T细胞淋巴瘤:2例未另行指定的外周T细胞淋巴瘤(PTL,NOS)、4例间变性大细胞淋巴瘤(ALCL)、1例T/NK细胞淋巴瘤。7例脂膜炎缺乏细胞学异型性,其特征为CD4 +和CD8 +细胞混合存在,其间散在L26 + B细胞聚集。仅偶尔有细胞显示TIA-1和穿孔素染色阳性。在T细胞淋巴瘤对照病例中,浸润倾向于累及真皮,有时甚至累及表皮,恶性细胞呈片状分布,这与SCPTCL中特征性的皮下定位和脂肪间隙边缘形成对比。2例PTL,NOS为CD4阳性,TIA-1和穿孔素均为阴性。尽管其余对照表达TIA-1和穿孔素,与其细胞毒性T或自然杀伤(NK)细胞起源一致,但组织学和其他免疫表型特征可将其与SCPTCL区分开来。5例SCPTCL还使用末端脱氧核苷酸转移酶介导的缺口末端标记试剂盒进行凋亡染色。所有病例均显示大量阳性凋亡小体,提示凋亡是这些肿瘤中细胞死亡的机制。我们的研究表明,SCPTCL构成一种独特的临床病理实体,起源于细胞毒性T淋巴细胞,应与其他累及皮下组织的良性和恶性淋巴样浸润相鉴别。这些肿瘤中所见的凋亡可能由细胞毒性颗粒蛋白的释放介导。