Kobayashi M, Yumiba C, Satoh T, Maruko T, Kishi T, Kawaguchi Y, Tanaka Y, Ueda K, Komazawa Y, Kaneda M
Department of Pediatrics, Hiroshima University School of Medicine, Japan.
Pediatr Res. 1989 Sep;26(3):246-9. doi: 10.1203/00006450-198909000-00018.
The sera from two patients with chronic neutropenia in infancy were examined for the presence of antineutrophil antibodies and their specificity against neutrophil antigen by using granulocyte indirect immunofluorescence test and microleukocyte agglutination test. In the microleukocyte agglutination test, the patients' sera reacted with neutrophils from their parents and normal unrelated donors having the neutrophil antigen NA1, but not with neutrophils from NA1- donors. After the absorption of patients' sera with NA1+ neutrophils, the antibody activity was completely abolished, resulting in the confirmation of the anti-NA1 antibody. In contrast, the granulocyte indirect immunofluorescence test showed positive reactions against both NA1+ and NA1- neutrophils, and the specificity for anti-NA1 was found in the results of the sera absorbed with NA1+ neutrophils. This suggested that the absorption experiment might be necessary to determine the specificity of the antibody for neutrophil antigen. Thus, we confirmed two cases with autoimmune neutropenia caused by anti-NA1 antibody. A combination of agglutination and immunofluorescence techniques would be recommended for investigation of neutrophil antibodies against the neutrophil-specific antigen.
采用粒细胞间接免疫荧光试验和微量白细胞凝集试验,检测了两名婴儿期慢性中性粒细胞减少症患者血清中抗中性粒细胞抗体的存在及其对中性粒细胞抗原的特异性。在微量白细胞凝集试验中,患者血清与具有中性粒细胞抗原NA1的其父母及正常无关供者的中性粒细胞发生反应,但与NA1阴性供者的中性粒细胞不发生反应。用NA1阳性中性粒细胞吸收患者血清后,抗体活性完全消失,从而证实了抗NA1抗体的存在。相比之下,粒细胞间接免疫荧光试验显示对NA1阳性和NA1阴性中性粒细胞均呈阳性反应,在用NA1阳性中性粒细胞吸收的血清结果中发现了抗NA1的特异性。这表明吸收试验对于确定抗体对中性粒细胞抗原的特异性可能是必要的。因此,我们确诊了两例由抗NA1抗体引起的自身免疫性中性粒细胞减少症病例。推荐采用凝集和免疫荧光技术相结合的方法来检测针对中性粒细胞特异性抗原的中性粒细胞抗体。