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

Authors

  • Joyshree Panda Consultant Pathologist, Anandalokesonoscan Centre, Siliguri, West Bengal, India
  • Swarnendu Pal Consultant Pathologist, Serampore Walsh Super Speciality Hospital, India
  • Riju Bhattacharya Demonstrator, Department of Pathology, MJN Medical College and Hospital, Coochbehar, West Bengal, India
  • Samira Kumar Behera Associate Professor, Department of Pathology, MKCG Medical College, Berhampur, Odisha, India
  • Subhasish Pati Assistant Professor, Department of PMR, North Bengal Medical College, Darjeeling, West Bengal, India

Keywords:

Hemophilia, Inhibitors, Recombinant factor, Bethesda.

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.

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Published

2022-01-17

How to Cite

Joyshree Panda, Swarnendu Pal, Riju Bhattacharya, Samira Kumar Behera, & Subhasish Pati. (2022). Prevalence of inhibitors in hemophilia patients and quantitative estimation of FVIII Inhibitors in hemophilia patients of Odisha. International Journal of Health and Clinical Research, 5(2), 871–874. Retrieved from https://www.ijhcr.com/index.php/ijhcr/article/view/4949