Kart L, Tor M, Altin R, Tekin I O, Sayarlůoglu H, Ustundag Y, Gun B D
Zonguldak Karaelmas University Medical Faculty Department of Pulmonology, 67600, Zonguldak/Turkey.
Monaldi Arch Chest Dis. 2003 Jan-Mar;59(1):84-7.
Primary immunodeficiency syndromes are rarely diagnosed among adults. Common variable immunodeficiency (CVID) is a congenital immunological disorder characterized by defective antibody production. In this report, we describe a 35-year-old male suffering from a common variable immunodeficiency, referred to us because of a lobar pneumonia. He had a history of recurrent pulmonary infections, which was present months before presentation, suggesting hypogammaglobulinemia. We found a severe hypogammaglobulinemia, which confirmed the diagnosis of CVID. His immunoglobulin profiles upon admission before infusion of immunoglobulin (normal ranges) were: IgG < 1.41 (8-17) g/l, IgA 0.25 (0.85-4.9) g/l, IgM 0.182 (0.5-3.7) g/l, and IgE < 2 (< 120) IU/ml. His HLA profiles were HLA A2 A26, B18 B38, Cw7, DR11 and DQ7 DQ9. He was treated with intravenous immunoglobulin. After this regimen, his IgG was maintained at > 6.0 g/L. On follow up, he has been free of opportunistic infections. In conclusion, CVID should be considered in the differential diagnosis of recurrent pneumonia in adults.
原发性免疫缺陷综合征在成人中很少被诊断出来。常见变异型免疫缺陷（CVID）是一种先天性免疫疾病，其特征是抗体产生缺陷。在本报告中，我们描述了一名35岁患有常见变异型免疫缺陷的男性，因大叶性肺炎转诊至我们这里。他有反复肺部感染的病史，在就诊前数月就已出现，提示低丙种球蛋白血症。我们发现了严重的低丙种球蛋白血症，这证实了CVID的诊断。他在输注免疫球蛋白前入院时的免疫球蛋白谱（正常范围）为：IgG < 1.41（8 - 17）g/l，IgA 0.25（0.85 - 4.9）g/l，IgM 0.182（0.5 - 3.7）g/l，IgE < 2（< 120）IU/ml。他的HLA谱为HLA A2 A26、B18 B38、Cw7、DR11和DQ7 DQ9。他接受了静脉注射免疫球蛋白治疗。经过这个疗程，他的IgG维持在> 6.0 g/L。在随访中，他没有发生机会性感染。总之，在成人反复肺炎的鉴别诊断中应考虑CVID。
