Lau Y L, Low L C, Jones B M, Lawton J W
Department of Paediatrics, University of Hong Kong, Queen Mary Hospital.
Clin Exp Immunol. 1991 Aug;85(2):202-8. doi: 10.1111/j.1365-2249.1991.tb05705.x.
We report a Chinese girl with the moderate phenotype of leucocyte adhesion deficiency (LAD), presenting with persistent omphalitis and recurrent soft tissue infections. She had subnormal adhesion-dependent neutrophil functions, such as chemotaxis and chemiluminescence response to a particulate stimulant (opsonised zymosan). Despite her adequate humoral response to documented herpes simplex virus type 1, parainfluenza type 2 and adenovirus infection in vivo, there was marked impairment in the generation of plaque-forming cells (PFC) driven by pokeweed mitogen (PWM) in vitro. IgM PFC were less severely affected than IgG and IgA PFC, probably because IgM production is less dependent on T cell help than IgA and IgG production. The patient's B cells and accessory cells had reduced function compared with the control subsets, while helper function of her CD4+ cells was virtually absent in the PWM-driven PFC assay. She also had marked defect in natural killer cell activity. The proliferation of her lymphocytes was normal to several plant lectins, including phytohaemagglutinin, concanavalin A and PWM, but markedly defective to OKT3.
我们报告了一名患有中度白细胞黏附缺陷（LAD）表型的中国女孩，她表现为持续性脐炎和复发性软组织感染。她的黏附依赖性中性粒细胞功能低于正常水平，如趋化性以及对颗粒刺激物（调理酵母聚糖）的化学发光反应。尽管她在体内对已记录的单纯疱疹病毒1型、副流感病毒2型和腺病毒感染有足够的体液反应，但在体外由商陆丝裂原（PWM）驱动的空斑形成细胞（PFC）生成方面存在明显损害。IgM PFC受到的影响不如IgG和IgA PFC严重，这可能是因为IgM的产生比IgA和IgG的产生对T细胞辅助的依赖性更小。与对照亚群相比，患者的B细胞和辅助细胞功能降低，而在PWM驱动的PFC试验中，她的CD4 +细胞的辅助功能几乎缺失。她的自然杀伤细胞活性也有明显缺陷。她的淋巴细胞对几种植物凝集素（包括植物血凝素、刀豆球蛋白A和PWM）的增殖反应正常，但对OKT3的反应明显缺陷。
