Muram-Zborovski Talia, Loeb Danielle, Sun Tsieh
Departments of Pathology, University of Colorado School of Medicine, Denver, CO 80220, USA.
Arch Pathol Lab Med. 2009 Jan;133(1):133-7. doi: 10.5858/133.1.133.
Intestinal T-cell lymphoma is a heterogenous group. These tumors differ in their association with enteropathy, intraepithelial or nonintraepithelial origin, primary or secondary involvement, and T-cell or natural killer-like T-cell immunophenotype. There are also nonneoplastic conditions, such as celiac disease, refractory sprue, and reactive T-cell infiltration that mimic intestinal T-cell lymphoma. Therefore, the differential diagnosis requires extensive morphologic, immunophenotypic, and molecular genetic studies. A subset of primary intestinal intraepithelial T-cell lymphoma has emerged in recent years that is distinguished from enteropathy-type T-cell lymphoma in terms of clinical presentation (nonenteropathic), morphology (monomorphic small to medium-sized cells), immunophenotype (CD3(-)CD8(+)CD56(+)), and cytogenetics. We report such a case with a unique immunophenotype (CD3(-), cytoplasmic CD3(+), CD4(-), CD8(+), CD5(-), CD7(+), CD16(-), CD56(+), CD57(-), CD103(+), T-cell intracellular antigen 1(+), and betaF1(+)) that is comparable to that of a newly identified subset of intraepithelial T cells. The tumor progressed rapidly and the patient died within 6 months after the onset of the disease. We recommend a large monoclonal antibody panel for the differential diagnosis of this heterogeneous group of T-cell lymphoma.
肠道T细胞淋巴瘤是一组异质性疾病。这些肿瘤在与肠病的关联、上皮内或非上皮内起源、原发性或继发性累及以及T细胞或自然杀伤样T细胞免疫表型方面存在差异。也有一些非肿瘤性疾病，如乳糜泻、难治性口炎性腹泻和反应性T细胞浸润，可模仿肠道T细胞淋巴瘤。因此，鉴别诊断需要广泛的形态学、免疫表型和分子遗传学研究。近年来出现了原发性肠道上皮内T细胞淋巴瘤的一个亚组，其在临床表现（非肠病性）、形态学（单形性小至中等大小细胞）、免疫表型（CD3(-)CD8(+)CD56(+)）和细胞遗传学方面与肠病型T细胞淋巴瘤不同。我们报告了这样一例具有独特免疫表型（CD3(-)、胞质CD3(+)、CD4(-)、CD8(+)、CD5(-)、CD7(+)、CD16(-)、CD56(+)、CD57(-)、CD103(+)、T细胞胞内抗原1(+)和βF1(+)）的病例，该免疫表型与新发现的上皮内T细胞亚组相当。肿瘤进展迅速，患者在疾病发作后6个月内死亡。我们建议使用大量单克隆抗体组合来鉴别诊断这一异质性T细胞淋巴瘤组。
