خراسان A در افراد هموفيل VIII الگوي مهاركننده فاكتور

Pattern of factor VIII in hibitor inpatiens with hemophilia A in Khorasan

Introduction: Nowadays with improvement of screening tests for blood donors, recombinant methods, intervention of molecular methods and virvcidal techniques, the risk of infection transmission by coagulation factor concentrates has been diminished. Whereas formation of factor VIII inhibitor is a new concern for the patients with hemophilia A. Patients with hemophilia who have inhibitor are capable for severe hemorrhage; their treatments are more difficult and expensive with long- time hospitalization and sometimes cause death. The aim of this study was to show existence of inhibitor in Northeastern Iran and to determine necessity of screening of patients with hemophilia A for factor VIII inhibitor all over the country. Method: About 140 registered patients with hemophilia A exist in Khorasan that with the help of Khorasan Hemophilia Center they were invited to Ghaem Hospital to answer a questionnaire. Among them, 102 patients with hemophilia refered to the hospital so that information and sampling were done. Their venous blood were mixed with 3.2 g/dl citrated sodium (0.109 M) and after preparation plasma, APTT Mix was done for all samples and then Bethesda test and dilution for determination titer of inhibitor were done. Results: Out of 102 patients with hemophilia A under survey, 20 (19.6%) had inhibitor and 82 patients (80.4%) were without inhibitor. Minimum and maximum titers of inhibitors were 0.8 and 6 B. U., respectively. None of patients with hemophilia A were assayed for inhibitor already. There was no significant relation between factor VIII inhibitor and age ofpatients (P=0. 712). Conclusion: As patients with hemophilia A in other countries, a number of patients with hemophilia A in Khorasan have inhibitor too (19.6%). Hence, screening of all patients and doing test every 6 months to follow up patients with inhibitor and detection new inhibitor in patients without ancient history of inhibitor are recommended.