Christopoulos Petros, Fisch Paul
Department of Thoracic Oncology, Thoraxklinik at the Heidelberg University Medical Center, Translational Lung Research Center Heidelberg (TLRC-H), Member of the German Center for Lung Research (DZL), Rontgenstr. 1, 69126 Heidelberg, Germany.
Department of Pathology, University Medical Center Freiburg, Breisacher Str. 115a, 79106 Freiburg, Germany.
Crit Rev Immunol. 2016;36(4):315-327. doi: 10.1615/CritRevImmunol.2017018916.
Acquired T-cell immunodeficiency can occur in thymoma patients with or without hypogammaglobulinemia (Good's syndrome), but it has received little attention to date. It appears predominantly associated with lymphocyte-rich (i.e., cortical or mixed) thymomas and frequently coexists with autoimmune manifestations. The main abnormalities are an increase in circulating naive T cells, cutaneous T-cell anergy, TCR hyporesponsiveness in vitro as well as a numerical and functional impairment of regulatory T cells. All of these probably result from an abnormal T-cell maturation in the neoplastic thymic microenvironment. A better understanding of thymoma-related acquired T-cell immunodeficiency will be important for immunotherapy of this orphan disease as well as for the prevention and treatment of opportunistic infections, autoimmune complications and secondary malignancies that contribute to the morbidity and mortality of thymoma patients.
获得性T细胞免疫缺陷可发生于有或无低丙种球蛋白血症（古德综合征）的胸腺瘤患者中，但迄今为止很少受到关注。它主要与富含淋巴细胞的（即皮质型或混合型）胸腺瘤相关，且常与自身免疫表现共存。主要异常包括循环中幼稚T细胞增加、皮肤T细胞无反应性、体外TCR低反应性以及调节性T细胞的数量和功能受损。所有这些可能都源于肿瘤性胸腺微环境中T细胞成熟异常。更好地理解胸腺瘤相关的获得性T细胞免疫缺陷对于这种罕见病的免疫治疗以及预防和治疗导致胸腺瘤患者发病和死亡的机会性感染、自身免疫并发症和继发性恶性肿瘤至关重要。
