Cuesta B, Fernández J, Pardo J, Páramo J A, Gómez C, Rocha E
Acta Haematol. 1986;75(1):1-5. doi: 10.1159/000206070.
A 10-year-old female with a complete selective IgA deficiency and recurrent autoimmune disease (chronic active hepatitis, focal glomerulonephritis, hemolytic anemia and thrombopenic purpura) is presented. Both serum IgA and saliva secretory IgA were below the detection limit. The small bowel biopsy using a peroxidase-antiperoxidase technique showed absence of plasma cells secreting IgA. Circulating antibodies against mitochondria, microsomal thyroid antigen were detected as well as rheumatoid factor. Circulating immune complexes were present. A positive Coombs' test and a slightly positive reaction for cryoagglutinins were demonstrated. No alterations in cellular immunity were observed. Clinical and analytical improvement with prednisone and azathioprine was obtained.
报告一名10岁女性，患有完全性选择性IgA缺乏症及复发性自身免疫性疾病（慢性活动性肝炎、局灶性肾小球肾炎、溶血性贫血和血小板减少性紫癜）。血清IgA和唾液分泌型IgA均低于检测限。采用过氧化物酶-抗过氧化物酶技术进行的小肠活检显示，缺乏分泌IgA的浆细胞。检测到抗线粒体、微粒体甲状腺抗原的循环抗体以及类风湿因子。存在循环免疫复合物。库姆斯试验呈阳性，冷凝聚素反应呈弱阳性。未观察到细胞免疫改变。使用泼尼松和硫唑嘌呤后临床及分析指标得到改善。
