Medical doctor Hemobiology and blood transfusion Annaba, Annaba, Algeria
Background: Hemophilia is a hereditary, life-threatening bleeding disorder characterized by a deficiency or dysfunction of clotting factors VIII (hemophilia A) or IX (hemophilia B), essential coagulation elements in the complex blood clotting cascade. One of the greatest complications in hemophilia is the development of inhibitor (alloantibodies) that inhibit FVIII or FIX activity.
Aims: We aimed to obtain data that are needed on the burden of inhibitors in our region to aid in prevention efforts.
Methods: Blood specimens were obtained from hemophilia subjects to be tested for FVIII and FIX antibodies. Inhibitor analysis was performed in the hemobiology-blood transfusion service at the Ibn Rochd hospital using a Bethesda assay.
Results: A total of 92 males with hemophilia A (HA) or hemophilia B (HB) were enrolled in the study. Data with complete baseline information were analyzed. Mean age was 28.24 (standard deviation (SD) =14.7) years, 69.54% were adults, 73.91% of patients were HA and 26.08% were HB. Approximately half of the patients (56%) have severe hemophilia. During the study period there were 10 cases of elevated inhibitor titers among 68 HA which represent 14.70% of cases and no case among HB participants. From the 10 patients who developed FVIII inhibitors, 6 have a high titer and 4 a low titer, two of whom had an inhibitor titer less than 1BU/ml. Two of the low-titer inhibitors were transient.
Conclusion(s): Our observed prevalence (14.7%) of Bethesda positive subjects is consistent with other studies performed on the same topic. These data will be useful in targeting populations for research and evaluating the effectiveness of public health prevention programs.
