A study on the pattern of inhibitor development in people with haemophilia A from Upper Assam, India

Abstract

Hemophilia is a X-linked recessive disorder of the coagulation cascade, that results in deficiency in clotting factors VIII and IX, with a prevalence of one in 5000 to one in 30,000 live births, in type A and B respectively. Factor replacement therapy remains the mainstay of treatment along with supportive measures but is often associated with development of alloantibodies against the transfused factors, resulting in suboptimal treatment response, resulting in more complications, increased expenditure and as a result compromising the quality of life. Methodology: In this hospital based cross sectional observational study done at the Hemophilia treatment centre in Assam Medical College and Hospital, Dibrugarh, India, 95 patients with Hemophilia A were enrolled. After taking due consent, detailed history was obtained and relevant investigations such as APTT (activated pro-thromboplastin time), Mixing test and Bethesda Assay were done. Results: Out of the 95 patients, 17.89% patients tested positive for inhibitors, with majority having severe disease (52.95%), followed by moderate disease (29.41%) and mild disease (17.64%). Five complications were reported in our study, with an exception of gum bleed, rest all were more in patients with inhibitors. The prevalence of inhibitors was found to be more among the recipients of recombinant product compared to plasma derived once. (58.82% vs 23.52%). Conclusion: With all the data, we concluded that, prevalence of inhibitor was more in severe forms of the disease, complications were more in PwHA with inhibitors and recipients of recombinant product were more likely to have inhibitors. Our study showed that 17.89% (n=17) PwHA developed inhibitors, which was more in age group, severity and after surgical procedures. This emphasizes the need for regular factor assessment and inhibitor screening as 1/5th of our patients developed inhibitor of which majority patients have low titre at our treatment centre.