Eosinophilia Associated with Disorders of Immune Deficiency or Immune Dysregulation.
Eosinophilia Associated with Disorders of Immune Deficiency or Immune Dysregulation.
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DOI：
10.1016/j.iac.2015.05.004
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发表时间：
2015-08
影响因子：
2.6
通讯作者：
Freeman AF
中科院分区：
文献类型：
--
作者：
Williams KW;Milner JD;Freeman AF
血清嗜酸性粒细胞水平升高与多种免疫缺陷或免疫失调有关。虽然原发性免疫缺陷疾病（PIDD）是罕见的，重要的是要考虑这些患者嗜酸性粒细胞增多的鉴别诊断。这篇综述讨论了几种免疫缺陷或失调疾病的临床特征、实验室结果、诊断和遗传基础，所有这些疾病都记录了嗜酸性粒细胞增多症作为该综合征的一部分。这篇文章包括常染色体显性高IgE综合征、DOCK8缺乏症、PGM3缺乏症、ADA-SCID、Omenn综合征、Wiskott-Aldrich综合征、Loeys-Dietz综合征、自身免疫性淋巴细胞增生性综合征、免疫失调、多内分泌病、肠病、x连锁综合征、Comel-Netherton综合征、严重皮炎、多重过敏和代谢消耗综合征（SAM）。
Elevated serum eosinophil levels have been associated with multiple disorders of immune deficiency or immune dysregulation. Although primary immunodeficiency diseases (PIDD) are rare, it is important to consider these in the differential diagnosis of patients with eosinophilia. This review discusses the clinical features, laboratory findings, diagnosis, and genetic basis of disease of several disorders of immune deficiency or dysregulation – all which have documented eosinophilia as part of the syndrome. The article includes autosomal dominant hyper IgE syndrome, DOCK8 deficiency, PGM3 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 (SAM).