Oyenuga Mosunmoluwa, Shaikh Safia, Harris Benjamin, Sinha Jyotsana, Lacasse Alexandre
Department of Internal Medicine, SSM St. Mary's Hospital, St. Louis, MO, USA.
Department of Hematology/Oncology, SSM St. Mary's Hospital, St. Louis, MO, USA.
J Community Hosp Intern Med Perspect. 2021 Mar 23;11(2):263-265. doi: 10.1080/20009666.2021.1877400.
Good syndrome (GS) is a rare paraneoplastic syndrome seen before or after diagnosis of thymoma, and its treatment, and is characterized by hypogammaglobulinemia. Rarely, pure white cell aplasia (PWCA) can also be seen which can present as recurrent neutropenia. We describe a 64-year-old man with recurrent sinus infections and previous thymectomy for stage 1 type B2 thymoma presenting with chronic diarrhea and recurrent neutropenia necessitating serial hospitalizations despite repeated antimicrobial treatment. Immunoglobulin levels, including IgM, IgA, IgD, and IgE were undetectable. Flow cytometry also showed absent B cells. Patient was initiated on immunoglobulin replacement therapy with consequent significant clinical improvement. Despite thymectomy, patients can develop thymoma-associated paraneoplastic syndromes, including GS.
古德综合征(GS)是一种罕见的副肿瘤综合征,可在胸腺瘤诊断之前或之后出现,其特征为低丙种球蛋白血症。罕见情况下,还可见纯白细胞再生障碍(PWCA),可表现为复发性中性粒细胞减少。我们描述了一名64岁男性,既往因1期B2型胸腺瘤行胸腺切除术,现反复出现鼻窦感染,伴有慢性腹泻和复发性中性粒细胞减少,尽管反复进行抗菌治疗仍需多次住院。免疫球蛋白水平,包括IgM、IgA、IgD和IgE均检测不到。流式细胞术也显示B细胞缺失。患者开始接受免疫球蛋白替代治疗,随后临床症状显著改善。尽管进行了胸腺切除术,患者仍可发生与胸腺瘤相关的副肿瘤综合征,包括古德综合征。