This study was purposed to investigate the diagnosis, typing and influencing factors of the antibody (inhibitor) to coagulation factors in hemophilia. 500 hemophilia patients were enrolled in this study. The activities of coagulation factor FVIII and FIX were tested by one stage assay. The antibodies of FVIII and FIX were detected by Bethesda assay. All data were analyzed by statistical soft SPSS 10.0. The results indicated that there were 411 cases of hemophilia A, out of which 151 cases (30.2%) showed FVIII antibody positive, the titer was 3.50 ± 2.84 Bu/ml; there were 79 cases of hemophilia B, out of which 18 cases (3.6%) showed FIX antibody positive, the titer was 2.92 ± 2.19 Bu/ml. The other 10 cases were acquired autogeneic hemophilia (2.0%). The antibody was divided into three types: high-response (3 cases), intermediate-response (47 cases), and low-response (119 cases). Among the 169 cases with antibody positive, 157 cases (92.9%) were younger than 30 years old; among 151 (89.35%) cases of hemophilia A; 138 cases (81.66%) were moderate or severe hemophilia; 166 case (98.22%) showed intermediate or low-response antibody. There were 158 cases with allogeneic antibody positive, all of which received blood transfusion. It is concluded that the moderate and low responsive antibodies are the dominant in hemophilia patients, the age of patients and transfusion frequency of blood preparation are the influencing factors. The results of this study provide the basis for the hemophilia diagnosis, antibody typing and evaluation of factors influencing hemophilia, and also suggest that the repeated transfusion of blood preparation may influence the production of antibodies.
Adolescent ; Adult ; Aged ; Autoantibodies ; blood ; Child ; Child, Preschool ; Factor VIII ; immunology ; Female ; Hemophilia A ; blood ; diagnosis ; immunology ; Humans ; Infant ; Male ; Middle Aged ; Young Adult