Prevalence of inhibitors in hemophilia patients and quantitative estimation of FVIII Inhibitors in hemophilia patients of Odisha

Abstract

Objective:To know prevalence of factor VIII and IX inhibitors in Haemophilia patients and Quantitative estimation of factor VIII inhibitors in Haemophilia A patients using Bethesda Assay. Study Population: Hemophilia Patients receiving blood products and recombinant factor transfusion. Results: Total 54 cases were screened for factor deficiency and inhibitors. 42 old cases were screened and 12 new cases of Hemophilia A were screened for development of inhibitors.The mean age of patients in the study population was 14.38+8.12 years with age ranging from 9 months to 68 years. Prevalence of Hemophilia A was 92.6%, prevalence of Hemophilia B was 5.5%. There were 46% severe hemophilia A cases and 44% moderate hemophilia A cases and 10% mild hemophilia A cases. 40.74% cases had development of target joints with knee joint which was most commonly effected. Prevalence of inhibitors in Hemophilia A was 8%.It was 13% in severe hemophilia cases. 25% i.e. 1 patient was high responder with inhibitor level of 64 BU, other 3 inhibitor positive patients were low responders with inhibitor levels of 3BU, 3.2 BU and 4.4 BU. Conclusion: Severe hemophilia patients need frequent factor transfusions and are at higher risk of inhibitor development. Patients with low inhibitor levels i.e.<10BU need high dose of recombinant factor VIII. Patients with high inhibitor levels >10 BU may require Recombinant factor VII with or without immune tolerance therapy. So inhibitor screening and Bethesda assay is needed at least once in every six months for prompt treatment.