title: [Expression of HLA-DRB1*15 genotype in children with acquired aplastic anemia and its relation to effect of immunosuppressive therapy].
abstract: This study was purpose to investigate the frequency of HLA-DRB1*15 expression in children with aplastic anemia (AA) and its relation to effect of immunosuppressive therapy. HLA-DR genotypes were detected by SSP-PCR in 40 patients with acquired aplastic anemia and 107 normal controls, and the expressions of HLA-DR gene in AA patients and normal controls were compared. 32 out of 40 patients were treated with immunosuppressive therapy, which included antilymphocyte globulin combining with cyclosporine or cyclosporine alone, the relation of HLA-DRB1*15 expression to efficacy of immunosuppressive therapy and relapse of AA was explored. The results showed that the mean age of the patients was 9.0 years with a ratio of male to female 1.5:1. The frequency of HLA-DRB1*15 genotype expression in patients with idiopathic aplastic anemia was 51.5% (17/33), which was markedly higher than that of healthy controls (20.6%, p<0.01). All of 7 patients with second acquired aplastic anemia showed negative expression of HLA-DRB1*15. The rates of all responses, including complete remission and partial remission (CR+PR), and CR to immunosuppressive therapy in 16 patients who bared HLA-DRB1*15 were 93.8% and 87.5% respectively, which were higher significantly than those of patients without bearing HLA-DRB1*15 (56.3% and 31.3%, p<0.01). Relapse occurred in 5 patients who bared HLA-DRB1*15 genotype. It is concluded that the frequency of HLA-DRB1*15 genotype expression in children with AA is significantly higher than that in normal controls, and the immunosuppressive therapy for patients bared HLA-DRB1*15 shows favourable effect with high incidence of complete remission.
authors: Department of Pediatrics, The Second Affiliated Hospital, SUN Yat-Sen University, Guangzhou 510120, China.
authors: Yong-Lan
authors: YL
authors: Huang
authors: Shao-Liang
authors: SL
authors: Huang
authors: Ke
authors: K
authors: Huang
authors: Rong
authors: R
authors: Bao
mesh_terms: D000293:Adolescent; D000741:Anemia, Aplastic / Q000188:drug therapy* / Q000235:genetics* / Q000276:immunology; D000961:Antilymphocyte Serum / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D016572:Cyclosporine / Q000627:therapeutic use; D005260:Female; D005838:Genotype; D006684:HLA-DR Antigens / Q000378:metabolism*; D059811:HLA-DRB1 Chains; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male
chemical_list: D000961:Antilymphocyte Serum; D006684:HLA-DR Antigens; D059811:HLA-DRB1 Chains; C507799:HLA-DRB1*15 antigen; D007166:Immunosuppressive Agents; D016572:Cyclosporine