[低γ -球蛋白血症和胸腺瘤(Good's综合征):1例报告和文献复习]。

Michela Di Renzo, Anna Laura Pasqui, Fulvio Bruni, Luca Voltolini, Giuseppe Gotti, Alberto Auteri
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引用次数: 0

摘要

古德氏综合征是一种罕见的成人免疫缺陷疾病,以低γ球蛋白血症和胸腺瘤为特征。一位61岁男性患者在2年复发性呼吸道感染史后被诊断为古德综合征。胸部x光和胸部计算机断层扫描显示手术切除的纵隔肿块。组织学显示胸腺瘤。手术后他出现了反复的呼吸道和尿路感染和食道念珠菌病,尽管在开始适当剂量的静脉注射免疫球蛋白治疗后,他的总体情况有了显著改善。实验室检查显示低γ -球蛋白血症,轻度中性粒细胞减少,淋巴细胞减少,无B细胞,CD4+淋巴细胞减少,CD4/CD8比例倒置,产生白细胞介素-4的CD4+淋巴细胞增加,提示Th2反应过度。
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[Hypogammaglobulinemia and thymoma (Good's syndrome): a case report and a literature review].

Good's syndrome is a rare adult-onset immunodeficiency disease characterized by hypogammaglobulinemia and thymoma. A 61-year-old male patient was diagnosed with Good's syndrome after a 2-year history of recurrent respiratory infections. Chest X-ray and chest computed tomography scan showed a mediastinal mass which was surgically removed. Histology revealed a thymoma. Following surgery he presented with recurrent respiratory and urinary tract infections and with esophageal candidiasis, even though his overall conditions dramatically improved after starting treatment with an appropriate dosage of intravenous immunoglobulins. Laboratory tests showed hypogammaglobulinemia, mild neutropenia, lymphopenia with no B cells, decreased CD4+ lymphocytes with an inverted CD4/CD8 ratio and increased interleukin-4-producing CD4+ lymphocytes, suggestive of an excessive Th2 response.

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