Beltran Brady E, Morales Domingo, Quinones Pilar, Miranda Roberto N, Goswami Maitrayee, Castillo Jorge J
Department of Oncology and Radiotherapy, Hospital Nacional Edgardo Rebagliati Martins, Lima, Peru.
Appl Immunohistochem Mol Morphol. 2012 Mar;20(2):196-200. doi: 10.1097/PAI.0b013e318225189f.
T-cell regulatory lymphocytes (T reg) are identified by their reactivity with CD4, CD25, and FOXP3, and are variably present in the background of various neoplasms including hematopoietic tumors, and function modulating the immune response, including control of autoimmunity. Adult T-cell leukemia/lymphoma is an aggressive lymphoma associated with human T-lymphotrophic virus 1 infection characterized by the presence of neoplastic lymphocytes with a T reg phenotype; however, this phenotype is not characteristically found in other lymphomas. Here, we report 2 apparently immunocompetent human T-lymphotrophic virus 1-negative patients with nodal and extranodal peripheral T-cell lymphoma, not otherwise specified with a T reg immunophenotype, based on the strong CD25 and FOXP3 positivity of the neoplastic cells. One patient was a 48-year-old woman with an early stage tumor in the cavum, who despite of chemotherapy subsequently developed systemic disease and died of tumor progression 46 months from diagnosis. The second patient was a 65-year-old male with generalized adenopathy and B symptoms who received chemotherapy achieving a complete remission but had recurrence and died 36 months from diagnosis. The histopathology revealed a diffuse infiltrate with an interfollicular distribution in the second case, with nodal involvement, consisted of large cells with clear cytoplasm associated with vascular proliferation and abundant mitoses. Neoplastic cells of first case showed typical T reg phenotype, whereas the second case had a CD4/CD8 double negative T reg variant. Only a single similar case was found in a review of the literature. We conclude that peripheral T-cell lymphoma, not otherwise specified with a T reg phenotype may represent a distinct category of T-cell lymphoma with an aggressive clinical course and poor prognosis.
T 细胞调节性淋巴细胞（Treg）可通过其与 CD4、CD25 和 FOXP3 的反应性来识别，并且在包括造血肿瘤在内的各种肿瘤背景中存在差异，其功能是调节免疫反应，包括控制自身免疫。成人 T 细胞白血病/淋巴瘤是一种与人类 T 淋巴细胞病毒 1 感染相关的侵袭性淋巴瘤，其特征是存在具有 Treg 表型的肿瘤性淋巴细胞；然而，这种表型在其他淋巴瘤中并不常见。在此，我们报告 2 例明显具有免疫活性的人类 T 淋巴细胞病毒 1 阴性患者，患有淋巴结和结外外周 T 细胞淋巴瘤，根据肿瘤细胞强烈的 CD25 和 FOXP3 阳性，诊断为未另行特指的具有 Treg 免疫表型的淋巴瘤。1 例患者为 48 岁女性，空洞内有早期肿瘤，尽管接受了化疗，但随后仍发展为全身性疾病，并在诊断后 46 个月死于肿瘤进展。第 2 例患者为 65 岁男性，有全身淋巴结肿大和 B 症状，接受化疗后达到完全缓解，但复发并在诊断后 36 个月死亡。组织病理学显示，第二例病例为弥漫性浸润，呈滤泡间分布，伴有淋巴结受累，由细胞质清晰的大细胞组成，伴有血管增生和大量有丝分裂。第一例病例的肿瘤细胞表现出典型的 Treg 表型，而第二例病例具有 CD4/CD8双阴性 Treg 变体。在文献综述中仅发现 1 例类似病例。我们得出结论，未另行特指的具有 Treg 表型的外周 T 细胞淋巴瘤可能代表一种具有侵袭性临床病程和不良预后的独特 T 细胞淋巴瘤类别。
