Fischer A
Département de Pédiatrie, INSERM U. 132, Hôpital Necker-Enfants-Malades, Paris, France.
Pathol Biol (Paris). 1992 Oct;40(8):789-92.
LFA-1, Mac-1, and p150,95 are leukocyte adhesion proteins called beta 2 integrins. LFA-1 is expressed at the surface of all leukocytes. A rare disease caused by mutations of the gene encoding the integrin beta 2 subunit is characterized by defective expression of these leukocyte adhesion molecules. Affected individuals have profound immune deficiency with severe bacterial infections for which the only effective treatment is bone marrow transplantation. These patients are unable to reject a transplant from only partially "Human Leucocyte Antigen" (HLA) compatible donors. In vivo studies have demonstrated decreased transplant rejection after injection of anti-LFA-1 antibodies to patients with other immune deficiencies. Simultaneous administration of an anti-CD2 antibody that blocks the second major pathway of lymphocyte adhesion can be expected to reduce even further the rate of rejection episodes.
淋巴细胞功能相关抗原-1（LFA-1）、巨噬细胞-1抗原（Mac-1）和P150，95是被称为β2整合素的白细胞粘附蛋白。LFA-1在所有白细胞表面表达。一种由编码整合素β2亚基的基因突变引起的罕见疾病，其特征是这些白细胞粘附分子的表达缺陷。受影响的个体有严重的免疫缺陷，伴有严重的细菌感染，唯一有效的治疗方法是骨髓移植。这些患者无法排斥来自仅部分“人类白细胞抗原”（HLA）相容供体的移植。体内研究表明，向患有其他免疫缺陷的患者注射抗LFA-1抗体后，移植排斥反应降低。同时给予一种阻断淋巴细胞粘附第二主要途径的抗CD2抗体，有望进一步降低排斥反应的发生率。
