Litzman J, Burianova M, Thon V, Lokaj J
Dept. Clin. Immunology, Masaryk University, Faculty Hospital, Brno, Czech.
Allergol Immunopathol (Madr). 1996 Jul-Aug;24(4):174-6.
A case report of a 16 year old boy in whom selective IgA deficiency progressed to typical common variable immunodeficiency (CVID) is described. This boy with a history of frequent but not severe respiratory tract infections was referred to hospital because of severe pleuropneumonia and decreased levels of IgA (0.23 g/L), but normal IgG and IgM levels. Lymphocyte subpopulation determination revealed a decreased proportion of CD4+ lymphocytes (30%) and an increased proportion of CD8+ lymphocytes (32%), while CD3+, CD19+ and CD16+/56+ subpopulations were normal. During the subsequent 17 months a gradual decrease in IgG (ultimate level 2.23 g/L), IgA (< 0.05 g/L) and IgM (< 0.05 g/L) levels was observed, the decrease in IgM being the slowest reflecting a constant heavy chain gene order on chromosome 14. The observation supports the thesis of a close relation of selective IgA deficiency and common variable immunodeficiency.
本文描述了一例16岁男孩的病例报告，该男孩的选择性IgA缺乏症进展为典型的普通变异型免疫缺陷病（CVID）。该男孩有频繁但不严重的呼吸道感染病史，因严重的胸膜肺炎和IgA水平降低（0.23 g/L）而被转诊至医院，但其IgG和IgM水平正常。淋巴细胞亚群测定显示CD4+淋巴细胞比例降低（30%），CD8+淋巴细胞比例升高（32%），而CD3+、CD19+和CD16+/56+亚群正常。在随后的17个月中，观察到IgG（最终水平2.23 g/L）、IgA（<0.05 g/L）和IgM（<0.05 g/L）水平逐渐下降，IgM下降最慢，这反映了14号染色体上恒定的重链基因顺序。该观察结果支持了选择性IgA缺乏症与普通变异型免疫缺陷病密切相关的论点。
