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Eosinophilia Associated with Disorders of Immune Deficiency or Immune Dysregulation

机译:嗜酸性粒细胞增多症与免疫缺陷或免疫调节异常有关

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摘要

Increased serum eosinophil levels have been associated with multiple disorders of immune deficiency or immune dysregulation. Although primary immunodeficiency diseases are rare, it is important to consider these in the differential diagnosis of patients with eosinophilia. In this review, the clinical features, laboratory findings, diagnosis, and genetic basis of disease of several disorders of immune deficiency or dysregulation are discussed. The article includes autosomal dominant hyper IgE syndrome, DOCK8 deficiency, phosphoglucomutase 3 deficiency, ADA-SCID, Omenn syndrome, Wiskott-Aldrich syndrome, Loeys-Dietz syndrome, autoimmune lymphoproliferative syndrome, immunodysregulation, polyendocrinopathy, enteropathy, X-linked syndrome, Comel-Netherton syndrome, and severe dermatitis, multiple allergies, and metabolic wasting syndrome.
机译:血清嗜酸性粒细胞水平升高与多种免疫缺陷或免疫调节异常有关。尽管原发性免疫缺陷疾病很少见,但在对嗜酸性粒细胞增多症患者进行鉴别诊断时,必须考虑这些因素。在这篇综述中,讨论了几种免疫缺陷或调节异常疾病的临床特征,实验室发现,诊断和疾病的遗传基础。文章包括常染色体显性遗传性高IgE综合征,DOCK8缺乏症,磷酸葡萄糖突变酶3缺乏症,ADA-SCID,Omenn综合征,Wiskott-Aldrich综合征,Loeys-Dietz综合征,自身免疫性淋巴增生性综合征,免疫失调,多内分泌病,肠病,X连锁综合征,Comel- Netherton综合征,严重的皮炎,多种过敏和代谢消耗综合征。

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