与免疫缺陷或免疫失调疾病相关的嗜酸性粒细胞增多症。
Eosinophilia Associated with Disorders of Immune Deficiency or Immune Dysregulation.
作者信息
Williams Kelli W, Milner Joshua D, Freeman Alexandra F
机构信息
Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, 33 North Drive, Building 33, Room 2W10A, Bethesda, MD 20892, USA.
Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, 10 Center Drive, Building 10/CRC, Room 5-3950, Bethesda, MD 20892, USA.
出版信息
Immunol Allergy Clin North Am. 2015 Aug;35(3):523-44. doi: 10.1016/j.iac.2015.05.004.
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.
血清嗜酸性粒细胞水平升高与多种免疫缺陷或免疫失调疾病相关。虽然原发性免疫缺陷病很少见，但在嗜酸性粒细胞增多患者的鉴别诊断中考虑这些疾病很重要。在本综述中，讨论了几种免疫缺陷或失调疾病的临床特征、实验室检查结果、诊断及疾病的遗传基础。本文包括常染色体显性高IgE综合征、DOCK8缺陷、磷酸葡萄糖变位酶3缺陷、腺苷脱氨酶严重联合免疫缺陷、奥门综合征、威斯科特-奥尔德里奇综合征、洛伊斯-迪茨综合征、自身免疫性淋巴增生综合征、免疫失调、多内分泌腺病、肠病、X连锁综合征、科梅尔-内瑟顿综合征以及严重皮炎、多种过敏和代谢消耗综合征。
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