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白细胞硷性磷酸酶(LAP)是正常人中性分叶白细胞的成份之一,其功能尚未清楚,也未能肯定LAP与杀菌作用有关。作者报道一名患反复细菌感染,同时伴有湿疹和免疫球蛋白E增多以及原发性白细胞硷性磷酸酶缺乏的病例。经过临床和实验室检查并未发现患者同时患有能引起LAP减低的疾病,如遗传性低磷酸酶血症、进行性肌萎缩、肾病综合征、慢粒白血病和阵发性睡眠性血红蛋白尿症等。患者血中这些缺乏LAP的中性白细胞的形态正常,核分叶正常,嗜天青颗粒的数目、特殊性颗粒的数目和超微结构并无异常。这些白细胞的趋向性活力和细胞的代谢过程也正常。体外实验证明,这些白细胞杀菌能力有轻微
Leukocyte Alkaline Phosphatase (LAP) is one of the components of normal human neutrophil leukocytes. The function of LAP is not yet clear, and it is not certain that LAP is related to bactericidal effect. The authors report a case of recurrent bacterial infections accompanied by an increase in eczema and immunoglobulin E and a deficiency in primary white blood cell alkaline phosphatase. After clinical and laboratory tests did not find that patients also suffer from diseases that can cause a decrease in LAP, such as hereditary hypophosphatasia, progressive amyotrophy, nephrotic syndrome, CML, and paroxysmal nocturnal hemoglobinuria Wait. These LAP-deficient neutrophils were normal in normal blood cells, with normal nuclear lobes, no abnormalities in the number of azurophilic granules, the number of special granules and the ultrastructure in patients’ blood. The leukocyte tropism and cellular metabolism are also normal. In vitro experiments show that these leukocytes bactericidal ability is slight