Title:Is the Detection of Factor IX Inhibitors in Hemophilia B Orphan than Factor VIII Inhibitors in Hemophilia A? A Concise, Systematic Review
Volume: 20
Issue: 3
Author(s): Hassan Mansouritorghabeh*Seyedeh T. Mohades
Affiliation:
- Immunology Research Center, Inflammation and Inflammatory Diseases Division, Mashhad University of Medical Sciences, Mashhad,Iran
Keywords:
Hemophilia A, hemophilia B, factor VIII inhibitors, factor IX inhibitors, quality of life, lyophilized coagulation.
Abstract:
Objective: Development of inhibitors in hemophilia A and B comprise significant
challenge for patients, hematologists, and health provider systems. It has recommended by the
World Federation of Hemophilia (WFH) to check inhibitors every 3-4 months. The incidence of
inhibitor in hemophilia B is lower than hemophilia A. Here, it tried to unravel whether the detection
of inhibitors in hemophilia B neglected compared to hemophilia A or not?
Methods: A comprehensive review carried out using six international and local medical search
engines on published contributions about inhibitors in hemophilia A and B in Iran.
Results: From 699 titles, 12 relevant papers were selected. The mean of factor VIII inhibitors in
hemophilia A was 14.8%. The mean of factor IX inhibitors in hemophilia B was 6%. The minimum and
maximum reported percentages of factor VIII inhibitors were 4% and 19.6%, while the minimum
and maximum of reported percentages of factor IX inhibitors were 0% and 11.8%, respectively.
The inhibitors in hemophilia A had reported in 6 papers. One paper had covered the inhibitors in
hemophilia B. There were five papers on inhibitors in both hemophilia A and B. The comparison
between the reported patients showed that 3020 patients with hemophilia A and 314 patients with
hemophilia B had studied.
Conclusion: Consistent with the lower frequency of hemophilia B and the lower development of
inhibitors in hemophilia B compared to hemophilia A, it was concluded that hemophilia B had not
neglected in Iran. It seems to be rational that each country, check rates of detection of inhibitors in
hemophilia B to identify whether it has neglected or not.
