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一名反复感染的成年人原发性白细胞碱性磷酸酶缺乏症

Primary leucocyte alkaline phosphatase deficiency in an adult with repeated infections.

作者信息

Repine J E, Clawson C C, Brunning R D

出版信息

Br J Haematol. 1976 Sep;34(1):87-94. doi: 10.1111/j.1365-2141.1976.tb00177.x.

Abstract

An individual with repeated bacterial infections, eczema, hyperimmunoglobulin E, and a primary deficiency of leucocyte alkaline phosphatase is described. The Lap-deficient neutrophils from this patient had marginally deficient bactericidal activity particularly when challenged with high ratios of bacteria per neutrophil. Leucotactic, metabolic and morphologic features of the neutrophils from the patient were normal. Evidence is presented which contrasts this patient's condition with previously described primary or secondary deficiencies of LAP.

摘要

本文描述了一名患有反复细菌感染、湿疹、高免疫球蛋白E以及白细胞碱性磷酸酶原发性缺乏症的个体。该患者缺乏LAP的中性粒细胞杀菌活性略有不足,尤其是在受到每个中性粒细胞对应高比例细菌的刺激时。该患者中性粒细胞的趋化性、代谢和形态学特征均正常。文中提供的证据将该患者的病情与先前描述的原发性或继发性LAP缺乏症进行了对比。

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