Development Of Inhibitors In Hemophilia A Patients Receiving Different Treatment Options

Abstract

Introduction: Hemophilia A is the most common inherited bleeding disorder in Pakistan. Apart from frequent bleeds and joint deformities, the formation of inhibitors is the major complication of hemophilia. Inhibitors are antibodies formed against the coagulation factor. The purpose of this study is to determine the incidence of inhibitor development in hemophilia A patient receiving different treatment modalities namely fresh frozen plasma (FFP)/cryoprecipitate, plasma derived FVIII concentrates and long-acting recombinant FVIII concentrates.

Aims & Objectives: To assess the development of inhibitors in hemophilia A patients receiving treatment modalities. Place and Duration of Study: The study was conducted at Chughtai Institute of Pathology from November 2021 to November 2023.

Material &Methods: A total of 75 registered patients of the Hemophilia Patient Welfare Society, Lahore and Sundas Foundation were initially screened for FVIII inhibitors. Those who were found negative for these inhibitors were included in the study and then divided into three equal groups of 25 patients each. One group received only FFP/cryoprecipitate for treatment, the other group received plasma derived FVIII concentrates and the last group received recombinant factor VIII concentrates. These patients were observed for two years i.e after 15-20 exposures and following this period their inhibitors screening was done again to establish the effect of different treatment modalities on inhibitor development. The data collected was entered and analyzed by SPSS-21, a p-value of ?0.05 was considered significant.

Results: Our study showed that only 3 patients out of 25, receiving FFP as treatment modality developed inhibitors. Whereas, none of the patients receiving plasma derived FVIII concentrates and recombinant factor VIII concentrates developed inhibitors. The P value calculated is 0.044 which is significant.

Conclusion: Plasma derived FVIII concentrates and recombinant FVIII concentrates are safer as compared to FFP/Cryoprecipitate and have lesser risk for inhibitor development.