Taniuchi S, Masuda M, Yamamoto A, Hasui M, Tsuji S, Komiyama Y, Takahashi H, Kobayashi Y
Department of Pediatrics, Kansai Medical University, Osaka, Japan.
Hum Immunol. 2001 Apr;62(4):408-13. doi: 10.1016/s0198-8859(01)00217-8.
We studied 18 children with autoimmune neutropenia (AIN) to evaluate whether there was a possible relationship between the specificity of granulocyte autoantibodies (anti-NA1,2) and the phenotype of the NA system. Direct granulocyte immunofluorescence test (D-GIFT) was positive in all patients, and indirect granulocyte immunofluorescence test (I-GIFT) was positive in 17 of these 18 patients, respectively. Fourteen of 18 patients showed preferential binding to neutrophils from NA(1+2-) phenotyped donors. Immunoblotting with anti-FcgammaRIIImAb showed that IgG prepared from 7 of 12 patients precipitated both FcgammaRIIIb from NA1 and NA2 neutrophil lysate, whereas the other 5 precipitated only NA1. Patients' IgG did not react with purified FcgammaRIIa. FcgammaRIIIb genotype were NA(1+2-) in 15 of 18 patients and NA(1+2+) in the other 3. FcgammaRIIa type of all patients were (H+R-). These distributions were significantly different from those of healthy Japanese blood donors (n = 608). The genotype of FcgammaRIIIb and FcgammaRIIa may affect the production of neutrophil specific auto-antibodies in AIN of infancy and influence its clinical course.
我们研究了18例自身免疫性中性粒细胞减少症（AIN）患儿，以评估粒细胞自身抗体（抗NA1,2）的特异性与NA系统表型之间是否存在可能的关系。所有患者的直接粒细胞免疫荧光试验（D-GIFT）均为阳性，18例患者中的17例间接粒细胞免疫荧光试验（I-GIFT）也分别为阳性。18例患者中有14例显示与来自NA(1+2-)表型供体的中性粒细胞有优先结合。用抗FcγRIIImAb进行免疫印迹显示，12例患者中有7例制备的IgG沉淀了来自NA1和NA2中性粒细胞裂解物的FcγRIIIb，而另外5例仅沉淀了NA1。患者的IgG与纯化的FcγRIIa无反应。18例患者中15例的FcγRIIIb基因型为NA(1+2-)，另外3例为NA(1+2+)。所有患者的FcγRIIa类型均为（H+R-）。这些分布与健康日本献血者（n = 608）的分布有显著差异。FcγRIIIb和FcγRIIa的基因型可能影响婴儿期AIN中中性粒细胞特异性自身抗体的产生，并影响其临床病程。
