Herrera-Sánchez Diana Andrea, León-Pedroza José Israel, Vargas-Camaño María Eugenia, Castrejón-Vázquez María Isabel
Instituto Mexicano del Seguro Social, Centro Médico Nacional Siglo XXI, Hospital de Especialidades, Servicio de Alergia e Inmunología Clínica, Ciudad de México.
Rev Alerg Mex. 2017 Apr-Jun;64(2):235-240. doi: 10.29262/ram.v64i2.194.
Good's syndrome is an association of thymoma and immunodeficiency. The symptoms are recurrent sinopulmonary infections in addition to the compressive side of thymoma. A laboratory finding is notable for the absence or decrease of B lymphocytes, hypogammaglobulinemia, inversion ratio CD4/CD8 and abnormal proliferative response to mitogens.
Female, 49-year-old started five months earlier with lower limb edema, postprandial vomiting, dysphagia, chronic diarrhea and weight loss. A second endoscopy ruled gastric neoplasia. Chest radiography with mediastinal widening, Thoraco-abdominal CT with bilateral pleural effusion and a mass in the anterior mediastinum, histopathological report of the tumor: B1 thymoma. Laboratory findings: IgG 349 mg/dL, IgA 70.3 mg/dL, 37.1 IgM mg/dL, Ca125 631 UI/mL, leukocytes 7890 mm3, hemoglobin 13.2 g/dL, lymphocytes 2060 mm3, CD16+CD56+ 122 cells/µL, CD19 77 cells/µL, CD3 2052 cells/µL, CD4 977 cells/µL, CD8 998 cells/µL; ratio CD4/CD8 0.98, hepatitis C, B and HIV negative. They requested valuation to Clinical Immunology and Allergy due to hypogammaglobulinemia, the diagnosis of Good's syndrome was confirmed and initiated with intravenous gamma globulin replacement to immunomodulatory dose of 1 g/kg, she reached replacement goal in the third dose of immunoglobulin intravenous, with clinical improvement. She died four months later from cardiac complications.
Despite the variability of presentation, Good's syndrome should be suspected as part of the paraneoplastic manifestations of thymoma.
古德综合征是胸腺瘤与免疫缺陷的一种关联。症状除了胸腺瘤的压迫症状外，还有反复的鼻窦肺部感染。实验室检查结果显著表现为B淋巴细胞缺失或减少、低丙种球蛋白血症、CD4/CD8倒置率以及对有丝分裂原的增殖反应异常。
一名49岁女性，五个月前开始出现下肢水肿、餐后呕吐、吞咽困难、慢性腹泻和体重减轻。第二次内镜检查排除了胃部肿瘤。胸部X线显示纵隔增宽，胸腹CT显示双侧胸腔积液以及前纵隔有一个肿块，肿瘤组织病理学报告：B1型胸腺瘤。实验室检查结果：IgG 349mg/dL，IgA 70.3mg/dL，IgM 37.1mg/dL，Ca125 631UI/mL，白细胞7890/mm³，血红蛋白13.2g/dL，淋巴细胞2060/mm³，CD16+CD56+ 122个细胞/µL，CD19 77个细胞/µL，CD3 2052个细胞/µL，CD4 977个细胞/µL，CD8 998个细胞/µL；CD4/CD8比值0.98，丙型肝炎、乙型肝炎和HIV均为阴性。由于低丙种球蛋白血症，患者被转至临床免疫学和过敏科评估，确诊为古德综合征，并开始静脉注射免疫调节剂量为1g/kg的丙种球蛋白进行替代治疗，在第三次静脉注射免疫球蛋白时达到替代目标，临床症状有所改善。四个月后，患者死于心脏并发症。
尽管临床表现存在差异，但古德综合征应被怀疑为胸腺瘤副肿瘤表现的一部分。
