[Respiratory manifestations associated with cyclic neutropenia and innate immune deficiency (IgG3 deficiency): a case report].

Daniel Alejandro Rodríguez-Flores, María Del Pilar Ortiz-Jiménez
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Abstract

Background: Inborn errors of immunity are a heterogeneous group of diseases caused by a diminished or absent response of one or more components of the immune system. IgG subclass deficiency is more common in childhood, especially in boys than in girls. IgG2 deficiency is the most common, followed by IgG3 deficiency.

Case report: A male pediatric patient experienced respiratory distress of viral and bacterial origin and asthma in his early years, with frequent emergency room admissions and/or hospitalizations. At age 5, he was diagnosed with cyclic neutropenia, so the Pediatric Hematology Department decided to offer conservative treatment. At age 6, he was evaluated by an Otorhinolaryngologist, who diagnosed allergic rhinitis and performed a septumplasty and turbinectomy. At age 7, he was evaluated by an Allergist, with normal laboratory results: immunoglobulin profile, lymphocyte subpopulations, and blood count. Subcutaneous allergen-specific testing was positive. Treatment with subcutaneous immunotherapy was initiated. At age 9, the patient relapsed, with symptoms of upper respiratory tract infection and asthma. Laboratory studies revealed IgG3 deficiency (7.81 mg/dL), so treatment with intravenous immunoglobulin was initiated.

Conclusion: The administration of human immunoglobulin and monoclonal antibodies is associated with a reduction in respiratory symptoms in patients with inborn errors of immunity and a better quality of life.

[与循环中性粒细胞减少症和先天免疫缺陷(IgG3缺乏)相关的呼吸症状:1例报告]。
背景:先天性免疫错误是由免疫系统的一种或多种成分的反应减少或缺失引起的一组异质性疾病。IgG亚类缺乏在儿童期更为常见,尤其是男孩比女孩更常见。最常见的是IgG2缺乏,其次是IgG3缺乏。病例报告:一名男性儿科患者在他的早期经历了病毒和细菌起源的呼吸窘迫和哮喘,频繁的急诊室入院和/或住院。5岁时,他被诊断为循环中性粒细胞减少症,因此儿科血液科决定提供保守治疗。6岁时,他接受了耳鼻喉科医生的检查,诊断为过敏性鼻炎,并进行了鼻中隔成形术和鼻甲切除术。7岁时,他接受了过敏症专科医生的检查,实验室结果正常:免疫球蛋白谱、淋巴细胞亚群和血细胞计数。皮下过敏原特异性试验阳性。开始皮下免疫治疗。9岁时复发,出现上呼吸道感染和哮喘症状。实验室研究显示IgG3缺乏(7.81 mg/dL),因此开始静脉注射免疫球蛋白治疗。结论:人免疫球蛋白和单克隆抗体的使用与先天性免疫缺陷患者呼吸道症状的减轻和生活质量的提高有关。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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