Inano Tadaaki, Yasuda Hajime, Tsukune Yutaka, Tsutsui Miyuki, Wali Nadila, Saeki Harumi, Kajino Kazunori, Hino Okio, Masaki Yasufumi, Komatsu Norio
Department of Hematology, Juntendo University School of Medicine, Tokyo, Japan.
Department of Pathology and Oncology, Juntendo University School of Medicine, Tokyo, Japan.
Case Rep Oncol. 2022 Jan 10;15(1):7-11. doi: 10.1159/000521463. eCollection 2022 Jan-Apr.
TAFRO syndrome is a relatively new disease entity first reported in 2010. We report a case of TAFRO syndrome accommodated by abnormal exacerbation of moderately differentiated gastric adenocarcinoma. The pathophysiology of TAFRO syndrome is largely unknown, but because the disease often responds to immunosuppressive therapy and also because T follicular helper (Tfh) cells are reported to be drastically decreased in TAFRO syndrome, involvement of a dysregulated immune system can be speculated. Growing evidence points toward a pivotal role of Tfh cells in tumor immunity through supporting ectopic lymphoid structures, which are recruitment sites for cells directly engaging in antitumor activity such as CD8 T cells, NK cells, and macrophages. In fact, Tfh cells are reported to positively correlate with longer survival in human colorectal and breast cancer. Combined with our observations of hyperprogressive gastric cancer in the presented patient, an impaired tumor immunity is strongly indicated in TAFRO syndrome.
TAFRO综合征是一种相对较新的疾病实体，于2010年首次报道。我们报告了一例由中度分化胃腺癌异常加重所导致的TAFRO综合征病例。TAFRO综合征的病理生理学在很大程度上尚不清楚，但由于该疾病通常对免疫抑制治疗有反应，并且据报道TAFRO综合征中T滤泡辅助（Tfh）细胞急剧减少，因此可以推测免疫系统失调与之有关。越来越多的证据表明，Tfh细胞通过支持异位淋巴结构在肿瘤免疫中起关键作用，而异位淋巴结构是直接参与抗肿瘤活动的细胞（如CD8 T细胞、NK细胞和巨噬细胞）的募集位点。事实上，据报道Tfh细胞与人类结直肠癌和乳腺癌的较长生存期呈正相关。结合我们对该患者中胃癌超进展的观察结果，强烈提示TAFRO综合征中存在肿瘤免疫受损。
