同种型缺陷(IgG 亚类和选择性 IgA、IgM、IgE 缺陷)。

Kamran Imam,Jenny Huang,Andrew A White
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引用次数: 0

摘要

背景:免疫球蛋白 G(IgG)亚类缺乏症和孤立的 IgA、IgM、IgE 缺乏症在文献中均有描述,发病率各不相同。方法:这些同种型缺乏症的表现各不相同,有的无症状,有的反复感染,对预防性抗生素产生抗药性。结果:特应性疾病和自身免疫性疾病是常见的合并症。IgE 缺乏与疫苗反应受损和恶性肿瘤风险增加有关,尤其是在无过敏性合并症的患者和非常见变异性免疫缺陷(CVID)体液免疫缺陷、IgM 缺乏、IgG2 缺乏和 CD4 淋巴细胞减少的患者中。结论对于这些高危患者,应着重考虑对恶性肿瘤进行密切监测。治疗方法多种多样,可能包括针对疾病的抗菌疗法和针对特定患者的预防性抗生素,对于难治性和复发性感染患者,可考虑使用免疫球蛋白替代治疗。
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Isotype deficiencies (IgG subclass and selective IgA, IgM, IgE deficiencies).
Background: Immunoglobulin G (IgG) subclass deficiencies and isolated IgA, IgM, IgE deficiencies have all been described in the literature with variable prevalence. Methods: These isotype deficiencies have a variable presentation from asymptomatic to recurrent infections resistant to prophylactic antibiotics. Results: Atopic disorders and autoimmune diseases are common comorbidities. IgE deficiency has been associated with impaired vaccine response and an increased risk of malignancy, particularly in patients with no allergic comorbidities and those with non-common variable immunodeficiency (CVID) humoral immunodeficiency, IgM deficiency, IgG2 deficiency, and CD4 lymphopenia. Conclusion: Close monitoring for malignancy should be strongly considered for these patients who are at risk. Treatment is variable and may include antimicrobial therapies for illnesses and prophylactic antibiotics in select patients, and immunoglobulin replacement can be considered for patients with refractory, recurrent infections.
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