Roy G, Pardo A, Leyva-Cobián F
Department of Immunology, Centro Ramón y Cajal, Madrid, Spain.
Acta Haematol. 1988;79(1):26-32. doi: 10.1159/000205685.
The phenotype and functions of monocytes in patients with haemophilia A and age-matched controls were studied. Fourteen male haemophiliacs were classified in three categories according to the mean number of units of factor VIII received during the last 5 years. Eleven patients were positive for antibodies to human immunodeficiency virus but none of our patients were homosexuals or drug abusers, nor do they fulfill the criteria of acquired immunodeficiency syndrome. Patients treated with high amounts of factor VIII concentrates (greater than 3 x 10(5) U/year) showed a significantly lower percentage of monocytes expressing HLA-DR, LFA-1 and CR3 antigens as compared with patients receiving lower amounts of factor VIII (less than 2 x 10(6) U/year) or controls. Kinetics of DR, LFA-1 and CR3 in cultured monocytes showed tht they were lost faster by monocytes from haemophiliacs treated with large amounts of factor VIII than by control monocytes. Adherence ability and chemotactic response of monocytes from patients treated with less than 3 x 10(5) U/year of factor VIII were also impaired. Although phagocytic indices were in normal ranges in haemophiliacs, a significant difference was observed between percentages of phagocytic monocytes from haemophiliacs treated with the largest doses of factor VIII and normal controls. Tests for respiratory burst activity, measured by chemiluminescence and superoxide anion generation, and Staphylococcus aureus killing were in normal ranges in haemophiliacs' monocytes.
对甲型血友病患者及年龄匹配的对照者的单核细胞表型和功能进行了研究。14名男性血友病患者根据过去5年接受的凝血因子Ⅷ单位平均数分为三类。11名患者人类免疫缺陷病毒抗体呈阳性,但我们的患者均非同性恋者或药物滥用者,也不符合获得性免疫缺陷综合征的标准。与接受较低剂量凝血因子Ⅷ(每年少于2×10⁶单位)的患者或对照者相比,接受大量凝血因子Ⅷ浓缩物治疗(每年大于3×10⁵单位)的患者表达HLA - DR、LFA - 1和CR3抗原的单核细胞百分比显著降低。培养单核细胞中DR、LFA - 1和CR3的动力学显示,接受大量凝血因子Ⅷ治疗的血友病患者的单核细胞比对照单核细胞更快地丧失这些抗原。每年接受少于3×10⁵单位凝血因子Ⅷ治疗的患者的单核细胞的黏附能力和趋化反应也受损。尽管血友病患者的吞噬指数在正常范围内,但接受最大剂量凝血因子Ⅷ治疗的血友病患者的吞噬单核细胞百分比与正常对照之间存在显著差异。通过化学发光和超氧阴离子生成测量的呼吸爆发活性测试以及金黄色葡萄球菌杀伤测试在血友病患者的单核细胞中均在正常范围内。