文摘
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-FcγRIIImAb showed that IgG prepared from 7 of 12 patients precipitated both FcγRIIIb from NA1 and NA2 neutrophil lysate, whereas the other 5 precipitated only NA1. Patients IgG did not react with purified FcγRIIa. FcγRIIIb genotype were NA(1+2−) in 15 of 18 patients and NA(1+2+) in the other 3. FcγRIIa type of all patients were (H+R−). These distributions were significantly different from those of healthy Japanese blood donors (n = 608). The genotype of FcγRIIIb and FcγRIIa may affect the production of neutrophil specific auto-antibodies in AIN of infancy and influence its clinical course.