Pham H T, Chollet-Martin S, Hakim J
Nouv Rev Fr Hematol (1978). 1987;29(1):23-8.
Clinical and biological features of a recently recognized inherited syndrome are reported. This syndrome, Leukocyte Adherence Deficiency, is characterized by recurrent, life-threatening infections. The increased susceptibility to infectious agents is mainly related in the inability of the leukocytes to adhere to certain surfaces, because all adherence-related functions of the neutrophils that lead to bacterial killing are impaired. Adherence deficiency is due to moderate or severe deficiency of three structurally related glycoproteins (MO1, LFA-1 and gp 150,95). All three glycoproteins are heterodimers and share a common subunit whose absence is the cause of the disease.
本文报告了一种最近发现的遗传性综合征的临床和生物学特征。这种综合征，即白细胞黏附缺陷症，其特征为反复发生的、危及生命的感染。对感染病原体易感性增加主要与白细胞无法黏附于某些表面有关，因为导致细菌杀伤的中性粒细胞所有与黏附相关的功能均受损。黏附缺陷是由于三种结构相关糖蛋白（MO1、LFA - 1和gp 150,95）中度或严重缺乏所致。这三种糖蛋白均为异二聚体，并共享一个共同亚基，该亚基的缺失是疾病的病因。
