Ricevuti G, Mazzone A, Pasotti D, Fioravanti A, Uccelli E, Notario A
Recenti Prog Med. 1989 Oct;80(10):520-5.
This report summarizes the clinical and laboratory findings from a group of our patients and literature reviews from several families who have a genetic deficiency in three related leukocyte membrane surface antigens known as CR3,LFA-1, and p150,95. Each surface antigen has an identical beta-chain noncovalenty linked to one of three distinct alpha-chain types. Patients affected by this autosomal recessive disease have now been identified by several investigators. The patients have an increased susceptibility to bacterial infections that is similar to patients who have other types of neutrophil functional deficiencies or neutropenia. Laboratory tests have indicated that isolated neutrophils from these patients have two major types of functional deficiencies that probably contribute to their reduced ability to overcome bacterial infections. This review focuses exclusively on the phagocytic and cytotoxic abnormalities of neutrophils from these patients.
本报告总结了我们一组患者的临床和实验室检查结果，以及来自几个家族的文献综述，这些家族在三种相关的白细胞膜表面抗原（即CR3、LFA-1和p150,95）存在基因缺陷。每种表面抗原都有一条相同的β链，通过非共价键与三种不同的α链类型之一相连。几位研究人员现已鉴定出受这种常染色体隐性疾病影响的患者。这些患者对细菌感染的易感性增加，这与患有其他类型中性粒细胞功能缺陷或中性粒细胞减少症的患者相似。实验室检测表明，这些患者分离出的中性粒细胞存在两种主要类型的功能缺陷，这可能导致他们克服细菌感染的能力下降。本综述专门关注这些患者中性粒细胞的吞噬和细胞毒性异常。
