Jesenak Milos, Zelieskova Maria, Repko Miroslav, Banovcin Peter
Department of Pediatrics, Jessenius Faculty of Medicine in Martin, Comenius University in Bratislava, Martin, Slovakia.
Department of Pediatrics, Hospital in Poprad, Slovakia.
Cent Eur J Immunol. 2020;45(3):361-363. doi: 10.5114/ceji.2020.101269. Epub 2020 Nov 1.
DiGeorge syndrome (DGS) is a primary immunodeficiency disease characterized by multiple clinical features, including congenital heart defects, typical facial appearance, hypocalcemia, and immunodeficiency associated to thymic hypoplasia. A subset of patients with DGS may also have contemporary allergic diseases, possibly in the context of T cell dysregulation. Our work presents an unusual case of DGS in coincidence with severe allergic asthma successfully treated by humanized monoclonal anti-IgE antibody, omalizumab. Biological therapy with omalizumab is indicated as an add-on treatment for poorly controlled asthma in patients with severe persistent allergic asthma aged 6 years and above, who meet strict criteria. While data available from clinical trials suggest that omalizumab is generally well-tolerated, a little is known about its efficacy and tolerability in the context of underlying immunodeficiency. We reported for the first time that omalizumab could be safely effective in treatment of severe allergic asthma in patients with DGS, without modification of immunological parameters.
迪乔治综合征(DGS)是一种原发性免疫缺陷疾病,其特征为多种临床症状,包括先天性心脏缺陷、典型面容、低钙血症以及与胸腺发育不全相关的免疫缺陷。一部分DGS患者可能同时患有过敏性疾病,这可能与T细胞调节异常有关。我们的研究展示了一例不同寻常的DGS病例,该病例合并严重过敏性哮喘,通过人源化抗IgE单克隆抗体奥马珠单抗成功治愈。奥马珠单抗生物疗法适用于年龄6岁及以上、符合严格标准的严重持续性过敏性哮喘且哮喘控制不佳的患者的附加治疗。虽然临床试验数据表明奥马珠单抗总体耐受性良好,但对于其在潜在免疫缺陷背景下的疗效和耐受性了解甚少。我们首次报道,奥马珠单抗可安全有效地治疗DGS患者的严重过敏性哮喘,且不会改变免疫参数。