title: FcgammaRIII b and FcgammaRIIa polymorphism may affect the production of specific NA1 autoantibody and clinical course of autoimmune neutropenia of infancy.
abstract: 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.
authors: Department of Pediatrics, Kansai Medical University, Osaka, Japan. taniuchi@takaii.kmu.ac.jp
authors: S
authors: S
authors: Taniuchi
authors: M
authors: M
authors: Masuda
authors: A
authors: A
authors: Yamamoto
authors: M
authors: M
authors: Hasui
authors: S
authors: S
authors: Tsuji
authors: Y
authors: Y
authors: Komiyama
authors: H
authors: H
authors: Takahashi
authors: Y
authors: Y
authors: Kobayashi
mesh_terms: D017668:Age of Onset; D000918:Antibody Specificity / Q000276:immunology*; D015703:Antigens, CD / Q000235:genetics* / Q000276:immunology; D001323:Autoantibodies / Q000096:biosynthesis* / Q000097:blood / Q000276:immunology; D001324:Autoantigens / Q000276:immunology*; D001327:Autoimmune Diseases / Q000097:blood / Q000235:genetics / Q000276:immunology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D019085:Fluorescent Antibody Technique, Direct; D019084:Fluorescent Antibody Technique, Indirect; D005838:Genotype; D006801:Humans; D007223:Infant; D007519:Isoantigens / Q000276:immunology*; D008297:Male; D009503:Neutropenia / Q000097:blood / Q000235:genetics / Q000276:immunology*; D011110:Polymorphism, Genetic; D017452:Receptors, IgG / Q000235:genetics* / Q000276:immunology
chemical_list: D015703:Antigens, CD; D001323:Autoantibodies; D001324:Autoantigens; C080568:Fc gamma receptor IIA; D007519:Isoantigens; D017452:Receptors, IgG; C067704:neutrophil-specific antigen NA1, human; C072753:neutrophil-specific antigen NA2