Pattern of factor VIII inhibitors in patients with hemophilia A in the north east of Iran

Abstract

This survey was conducted to evaluate coagulation factor VIII:C inhibitors among 102 hemophilia A patients from different cities of Khorasan province in north east of Iran in order to identify and characterize the pattern of inhibitor formation in these patients population. For this purpose, we randomly obtained plasma samples of 102 hemophilia A patients (44 patients with severe, 28 patients with intermediate and 30 patients with mild hemophilia A) and studied them using two tests: the APTT mix and Bethesda test were performed. In the whole group 20 patients (19.6%) factor VIII inhibitors were detected. These were in 11 patients with severe, five patients with intermediate and four patients with mild hemophilia A. None of patients with hemophilia A had previously been studied for the presence of an inhibitor, so there was no existing history of inhibitor evaluation.

The prevalence of factor VIII and IX inhibitors among Saudi patients with hemophilia

“…[33] Nevertheless, these discrepancies may be related to differences in the study populations, management trends, and testing strategies. A study of 102 Iranian patients with hemophilia A (44 severe cases, 28 intermediate cases, and 30 mild cases) found that only 20 patients (19.6%) had inhibitors (11 severe cases, 5 intermediate cases, and 4 mild cases) [34] ; these findings are similar to our present findings. A large Indian study of 1285 patients with hemophilia A found that only 6.07% of the patients had inhibitors, although there were remarkable regional variations (the highest prevalence was 20.99%).…”

Hemophilia in Iran

“…Although Nijmegen assay is the recommended method for inhibitor assay by the International Society of Thrombosis and Hemostasis, most Iranian coagulation laboratories use the Bethesda assay. 15,19 Owing to the large size of FVIII, and to some extent FIX, genes, and genetic diversity in these genes, molecular diagnosis is not routinely performed for diagnosis or carrier detection in Iran. Genetic analysis imposes high costs on patients and the healthcare system.…”

Bleeding disorders and reduced bone density

“…Nowadays, long-term survival of individuals with haemophilia has been improved noticeably that attributable to many factors including improved treatment regimens, but new concerns such as inhibitor autoantibody to coagulation factors and RBD have been emerged recently [10,11]. We also need new insights into the pathophysiology and also management of reduced bone density in bleeding disorders.…”