Ottonello L, Dapino P, Pastorino G, Dallegri F, Sacchetti C
Department of Internal Medicine, University of Genova Medical School, Italy.
Eur J Clin Invest. 1995 Sep;25(9):687-92. doi: 10.1111/j.1365-2362.1995.tb01987.x.
A critical evaluation of 3 years' experience using laboratory screening to detect neutrophil dysfunction is described. Neutrophil dysfunctions in patients with recurrent bacterial infections were investigated by using the following screening tests: (1) neutrophil chemotaxis towards N-formylmethionyl peptides (FMLP) and the complement fragment C5a; (2) neutrophil production of superoxide anions (O2-) in response to phorbol myristate acetate and opsonized zymosan particles; and (3) examination of May-Grünwald and myeloperoxidase cytochemical staining of peripheral blood smears. These tests were carried out in 100 patients suffering from infections and suspected of having altered neutrophil functional competence. A minority of patients was found to have well defined neutrophil dysfunction syndromes: chronic granulomatous disease (four cases), Chediak-Higashi disease (one case) and myeloperoxidase deficiency (one case). Of the remaining 94 patients, in whom infections localized to airways and/or skin predominated, 53 cases were found to have impaired chemotaxis (41 cases) or partial defects of the O2- production. Defects of chemotaxis toward FMLP and those towards both FLMP and C5a were the most frequent abnormalities. No defect was found in the other 41 patients. Moreover, impaired neutrophil chemotaxis was found in some patients with selective IgA deficiency (five cases) or immotile cilia syndrome (seven cases). The results suggest that (a) additional screening tests are required to ameliorate the efficiency of the diagnostic work-up of the patients suspected to have neutrophil dysfunction; and (b) further evaluation, also at the molecular level, should be considered at least in selected cases of non-classified neutrophil dysfunction in order to clarify diagnosis and plan rational therapeutic strategies.
本文描述了对3年实验室筛查检测中性粒细胞功能障碍经验的批判性评估。通过以下筛查试验对复发性细菌感染患者的中性粒细胞功能障碍进行了研究：（1）中性粒细胞对N-甲酰甲硫氨酰肽（FMLP）和补体片段C5a的趋化作用；（2）中性粒细胞对佛波酯肉豆蔻酸酯乙酸盐和调理酵母聚糖颗粒产生超氧阴离子（O2-）的情况；（3）对瑞氏-吉姆萨染色和髓过氧化物酶细胞化学染色的外周血涂片进行检查。这些试验对100名患有感染且怀疑中性粒细胞功能能力改变的患者进行。发现少数患者患有明确的中性粒细胞功能障碍综合征：慢性肉芽肿病（4例）、切迪阿克-希格ashi病（1例）和髓过氧化物酶缺乏症（1例）。在其余94例以气道和/或皮肤局部感染为主的患者中，发现53例存在趋化功能受损（41例）或O2-产生部分缺陷。对FMLP的趋化缺陷以及对FMLP和C5a两者的趋化缺陷是最常见的异常情况。另外41例患者未发现缺陷。此外，在一些选择性IgA缺乏症（5例）或不动纤毛综合征（7例）患者中也发现了中性粒细胞趋化功能受损。结果表明：（a）需要额外的筛查试验来提高对怀疑有中性粒细胞功能障碍患者的诊断检查效率；（b）至少在未分类的中性粒细胞功能障碍的选定病例中，应考虑在分子水平上进行进一步评估，以明确诊断并制定合理的治疗策略。
