Fernández Romero Diego S, Juri María C, Paolini María V, Malbrán Alejandro
Unidad de Alergia, Asma e Inmunología Clínica, Hospital Británico de Buenos Aires, Argentina.
Medicina (B Aires). 2013;73(4):315-23.
Common variable immunodeficiency (CVID) is characterized by an impaired antibody production and an increased susceptibility to recurrent infections of the respiratory tract, mainly by extracellular encapsulated bacteria. We analyzed the clinical characteristics of 69 patients evaluated over a period of 10 years at three centers in the city of Buenos Aires. At the onset of the study 14 patients were on follow up, and at its end the number of patients reached to 60. Most of them consulted for infection or hypogammaglobulinemia and nearly half had an established diagnosis of immunodeficiency. Sixty-five (94.2%) patients had infections by encapsulated bacteria, four (6.1%) sepsis and two tuberculosis. The average age of onset of infectious symptoms was 18.1 years; the average age at diagnosis was 29.6 years and the delay to diagnosis 11.9 years. Forty one (59.4%) patients reported a history of recurrent or chronic diarrhea. In 22 (31.9%) 13 autoimmune diseases were diagnosed, being the most frequent the hematological disorders and hypothyroidism. Eight patients had histological polyclonal lymphoproliferation, four (5.8%) with granulomatous disease affecting the liver, the larynx and/or the skin; and four as lymphoid interstitial pneumonitis (LIP). Nineteen (27.5%) patients had splenomegaly and 23/57 (40.3%) images suggestive of lymphocytic or granulomatous processes (including the 4 with LIP) in the chest CT. Three (4.3%) patients developed B cell lymphoma, four (5.8%) stomach adenocarcinoma and one breast cancer. The study had a median follow-up of 54 months, range 1-353 and four patients (5.8%) died during the follow up.
普通可变免疫缺陷（CVID）的特征是抗体产生受损，以及对呼吸道反复感染的易感性增加，主要是由细胞外包膜细菌引起的。我们分析了在布宜诺斯艾利斯市三个中心10年间评估的69例患者的临床特征。研究开始时，14例患者正在接受随访，研究结束时患者人数达到60例。他们大多数因感染或低丙种球蛋白血症就诊，近一半患者已确诊免疫缺陷。65例（94.2%）患者有包膜细菌感染，4例（6.1%）发生败血症，2例患有结核病。感染症状的平均发病年龄为18.1岁；诊断时的平均年龄为29.6岁，诊断延迟时间为11.9年。41例（59.4%）患者报告有反复或慢性腹泻病史。22例（31.9%）患者被诊断出13种自身免疫性疾病，最常见的是血液系统疾病和甲状腺功能减退。8例患者有组织学上的多克隆淋巴细胞增殖，4例（5.8%）患有影响肝脏、喉部和/或皮肤的肉芽肿性疾病；4例为淋巴细胞间质性肺炎（LIP）。19例（27.5%）患者有脾肿大，胸部CT检查中23/57例（40.3%）有淋巴细胞或肉芽肿性病变（包括4例LIP患者）。3例（4.3%）患者发生B细胞淋巴瘤，4例（5.8%）发生胃腺癌，还有1例乳腺癌。该研究的中位随访时间为54个月，范围为1 - 353个月，4例（5.8%）患者在随访期间死亡。
