Tomooka Y, Torisu M, Miyazaki S, Goya N
J Clin Immunol. 1986 Sep;6(5):355-62. doi: 10.1007/BF00915374.
We treated a family with three children with histiocytosis X (H-X). The chemotactic response of the neutrophils in these three patients was depressed and the chemotactic response of the neutrophils of the mother was also depressed compared to that of normal age-matched controls. To elucidate the genetic factors, we examined HLA antigens in five members of this family. All five members had Aw24, B7, Cw7, and DR1. Immunological and genetic studies in an additional 32 patients with H-X were performed. The chemotactic response of 35 patients with H-X (154.9 +/- 58.4/HPF) was significantly depressed in comparison with that of 35 age-matched healthy controls (613.3 +/- 116.7/HPF). In addition, the value of chemiluminescence of 20 of 35 patients (20.5 +/- 6.6 mV) was also significantly depressed in comparison with that of 20 normal controls (45.3 +/- 11.4 mV). The frequencies of Bw61 (54.4%) and Cw7 (45.4%) in 33 patients with H-X were significantly increased in comparison with those of 250 normal healthy controls (20.4 and 18.0%, respectively). Studies of immunoglobulin levels and complement titers of patients with H-X showed no consistent abnormalities. We proposed that defects of polymorphonuclear function may lead to an increased susceptibility to bacterial infections in patients with this disorder.
我们治疗了一个有三个孩子患组织细胞增多症X（H-X）的家庭。与年龄匹配的正常对照组相比，这三名患者中性粒细胞的趋化反应降低，其母亲中性粒细胞的趋化反应也降低。为了阐明遗传因素，我们检测了这个家庭五名成员的HLA抗原。所有五名成员都有Aw24、B7、Cw7和DR1。对另外32例H-X患者进行了免疫学和遗传学研究。与35名年龄匹配的健康对照组（613.3±116.7/HPF）相比，35例H-X患者（154.9±58.4/HPF）的趋化反应明显降低。此外，35例患者中有20例（20.5±6.6 mV）的化学发光值与20名正常对照组（45.3±11.4 mV）相比也明显降低。与250名正常健康对照组（分别为20.4%和18.0%）相比，33例H-X患者中Bw61（54.4%）和Cw7（45.4%）的频率显著增加。对H-X患者免疫球蛋白水平和补体滴度的研究未发现一致的异常。我们提出，多形核功能缺陷可能导致该疾病患者对细菌感染的易感性增加。
