Leukocyte adhesion deficiency is an autosomal recessive primary immunodeficiency that has been divided into three types: LAD1 (beta-2 integrin (CD18) family deficiency/defect), LAD2 (absence of fucosylated carbonhydrate ligands for selectins) and LAD3 (defective activation of all beta integrins). However, recently LAD4 has been described in cystic fibrosis patients, with a defect in integrin activation reported in monocytes. LAD-I is the most common type and prevalence of 1 in 1,000,000 live births. Clinical features of LAD patients are recurrent bacterial and fungal infections, omphalitis with delayed umbilical stump separation, significant leukocytosis especially neutrophilia during infection periods, impaired pus formation, and delayed traumatic or surgical wound healing. Flow cytometry is considered a useful tool for rapid diagnosis of the disease. The study of CD18 and CD11 (a, b, c) expression patterns in peripheral blood leukocytes helps to distinguish different phenotypes of LAD-I. In general, patients with ≥ 2% CD18 expression tend to have a less severe infection and often survive until adulthood, whereas < 2% CD18 expression often results in death in infancy. In this case report, three siblings, 10, 15, and 17 years old, diagnosed with leukocyte adhesion defect type 1 in adolescence age group, are presented.

白细胞粘附缺陷是一种常染色体隐性原发性免疫缺陷，已分为三种类型：LAD1（β-2 整合素 (CD18) 家族缺陷/缺陷）、LAD2（缺乏用于选择素的岩藻糖基化碳水化合物配体）和 LAD3（所有 β 的缺陷激活整合素）。然而，最近在囊性纤维化患者中描述了 LAD4，在单核细胞中报告了整合素激活缺陷。LAD-I 是最常见的类型，每 1,000,000 名活产中就有 1 名患病率。LAD 患者的临床特征是复发性细菌和真菌感染、脐炎伴脐残端分离延迟、感染期间白细胞增多，尤其是中性粒细胞增多、脓液形成受损以及外伤或手术伤口愈合延迟。流式细胞术被认为是快速诊断疾病的有用工具。外周血白细胞中 CD18 和 CD11 (a, b, c) 表达模式的研究有助于区分 LAD-I 的不同表型。一般来说，CD18 表达≥ 2% 的患者感染的严重程度往往较轻，通常可以存活到成年，而 CD18 表达 < 2% 的患者通常会导致婴儿期死亡。在本病例报告中，介绍了三个兄弟姐妹，分别为 10、15 和 17 岁，在青春期被诊断为 1 型白细胞粘附缺陷。2% CD18 表达通常会导致婴儿期死亡。在本病例报告中，介绍了三个兄弟姐妹，分别为 10、15 和 17 岁，在青春期被诊断为 1 型白细胞粘附缺陷。2% CD18 表达通常会导致婴儿期死亡。在本病例报告中，介绍了三个兄弟姐妹，分别为 10、15 和 17 岁，在青春期被诊断为 1 型白细胞粘附缺陷。