Kitano Haruka, Yamaguchi Fumihiro, Atarashi Kenji, Hiraiwa Mina, Shiratori Yo, Onozaki Shota, Shikama Yusuke
Department of Respiratory Medicine, Showa University Fujigaoka Hospital, Yokohama, Kanagawa, 227-8501, Japan.
Onco Targets Ther. 2021 Oct 12;14:5045-5049. doi: 10.2147/OTT.S326193. eCollection 2021.
Good syndrome is a rare condition characterized by the presence of thymoma in combination with adult-onset hypogammaglobulinemia. Immunological features of Good syndrome include various immunodeficiencies accompanied with hypogammaglobulinemia. In patients with thymoma, paraneoplastic syndromes including hypogammaglobulinemia worsen the prognosis. We herein describe a patient with advanced-stage type A thymoma who was effectively treated with chemotherapy and exhibited a parallel decrease in the serum level of soluble interleukin-2 receptor (sIL-2R), which depends on cellular immunity. The present case suggests the efficacy of sIL-2R as a potential prognostic biomarker in a subset of patients with Good syndrome.
古德综合征是一种罕见病症,其特征为胸腺瘤合并成人期低丙种球蛋白血症。古德综合征的免疫学特征包括各种免疫缺陷并伴有低丙种球蛋白血症。在胸腺瘤患者中,包括低丙种球蛋白血症在内的副肿瘤综合征会使预后恶化。我们在此描述一名晚期A型胸腺瘤患者,该患者接受化疗后得到有效治疗,且其依赖细胞免疫的可溶性白细胞介素-2受体(sIL-2R)血清水平出现相应下降。本病例提示sIL-2R作为古德综合征部分患者潜在预后生物标志物的有效性。