Department of Pathology and Laboratory Medicine, All India Institute of Medical Sciences, Bhubaneswar, Odisha, 751019, India.
Department of Radiotherapy, All India Institute of Medical Sciences, Bhubaneswar, Odisha, 751019, India.
J Cancer Res Clin Oncol. 2021 Jan;147(1):295-301. doi: 10.1007/s00432-020-03440-2. Epub 2020 Nov 9.
Peripheral blood T lymphocytosis (PBTL) is a rare, yet poorly understood manifestations of thymoma, which is postulated to be linked with autoimmune/paraneoplastic manifestations such as myasthenia gravis (MG), pure red cell aplasia (PRCA), etc.; more commonly encountered in this neoplasm.
We aim to describe the flowcytometric immunophenotypic data of PBTL in a 43-year-old male; 6 months after successful completion of chemoradiotherapy (CT/RT) for a large, invasive, and metastatic type B1 thymoma; and present a comprehensive review of all such cases reported over last 42 years (N = 21) (1977-2019).
A larger size of the tumors (≥ 10 cm), presence of local invasion and/or distant metastasis, and type B (cortical or lymphocyte rich) histology were more likely to be associated with PBTL. Tumors associated with MG/PRCA (N = 9/21) tend to have lower PBTL compared to those without such manifestations; and PBTL subsided following thymectomy with or without CT/RT. Immunophenotypic analysis of PB revealed a CD8 + > CD4 + mature (naïve) polyclonal T cells resembling late cortical thymocytes.
Thymic intratumoral microenvironment might influence occurrence PBTL that may have a pathophysiologic link to development of autoimmune manifestations. Immunophenotypic characteristics of peripheral blood lymphoid cells should be the clue for accurate characterization and to avoid a misdiagnosis of a lymphoproliferative neoplasm.
外周血 T 淋巴细胞增多症(PBTL)是胸腺瘤的一种罕见但尚未被充分认识的表现形式,据推测与自身免疫/副肿瘤表现有关,如重症肌无力(MG)、纯红细胞再生障碍性贫血(PRCA)等;在这种肿瘤中更常见。
我们旨在描述一名 43 岁男性的 PBTL 的流式细胞免疫表型数据;在成功完成大侵袭性和转移性 B1 型胸腺瘤的放化疗(CT/RT)后 6 个月;并对过去 42 年来所有此类病例进行全面回顾(N=21)(1977-2019 年)。
肿瘤较大(≥10cm)、存在局部侵犯和/或远处转移、B 型(皮质或富含淋巴细胞)组织学更可能与 PBTL 相关。与 MG/PRCA 相关的肿瘤(N=9/21)与无此类表现的肿瘤相比,PBTL 倾向于更低;并且在进行胸腺切除术加或不加 CT/RT 后 PBTL 消退。PB 的免疫表型分析显示 CD8+>CD4+成熟(幼稚)多克隆 T 细胞类似于晚期皮质胸腺细胞。
胸内肿瘤微环境可能会影响 PBTL 的发生,这可能与自身免疫表现的发展存在病理生理联系。外周血淋巴细胞的免疫表型特征应该是准确特征描述的线索,并避免误诊为淋巴增生性肿瘤。