Krebs-Wurtz E, Mennecier B, Imler M
Service de Médecine interne, Hôpital de Hautepierre, Strasbourg.
Presse Med. 1998 Feb 28;27(8):351-3.
Common variable immunodeficiency (CVI) is a heterogeneous disorder characterized by decreased production of antibodies. Clinical presentation of CVI is generally that of recurrent pyogenic infections. Autoimmune diseases can also occur. The age of onset of symptoms shows two peaks at 1-5 and 16-20 years.
A 77-year-old man was admitted in hospital for pernicious anemia. We discovered hypogammaglobulinemia with low levels of immunoglobulin G, A and M, but normal T-cell levels. We diagnosed common variable immunodeficiency. This patient had not had recurrent pyogenic infections.
This case shows that common variable immunodeficiency can be revealed late by an autoimmune disease. The pathogenesis of autoimmune diseases in this immunodeficiency remains unknown despite several possible explanations.
普通可变免疫缺陷（CVI）是一种异质性疾病，其特征为抗体产生减少。CVI的临床表现通常为复发性化脓性感染。也可发生自身免疫性疾病。症状出现的年龄有两个高峰，分别在1 - 5岁和16 - 20岁。
一名77岁男性因恶性贫血入院。我们发现其存在低丙种球蛋白血症，免疫球蛋白G、A和M水平较低，但T细胞水平正常。我们诊断为普通可变免疫缺陷。该患者未曾有复发性化脓性感染。
本病例表明，普通可变免疫缺陷可由自身免疫性疾病较晚发现。尽管有几种可能的解释，但这种免疫缺陷中自身免疫性疾病的发病机制仍不清楚。
