“Prevalence of Inhibitors in Hemophilia Patients and its Clinical Implications”: A Study of 276 Patients in Western India

Abstract

Introduction: Hemophilia is an X-linked congenital bleeding disorder caused by a deficiency of coagulation factor VIII (FVIII) in hemophilia A (HA) or factor IX (FIX) in hemophilia B (HB). Accurate diagnosis of hemophilia by factor assay to demonstrate deficiency of FVIII or FIX is essential for appropriate management. Inhibitor development results in partial or complete lack of the efficacy of replacement therapy, and it makes the management of patients more difficult with an increased risk of morbidity, serious bleeding, and disability, resulting in a substantial impact on patient's quality of life and health-care costs, compared to patients without inhibitors. Aims and Objectives: To assess the incidence of inhibitor development in HA and HB patients along with its consequences. Materials and Methods: The present study was carried out at a tertiary care teaching hospital in Western India. A total of 276 patients of hemophilia were included in the study. FVIII, FIX, and inhibitor screening were carried out in all patients sample as routine testing. Patients who were found positive in inhibitor screening were further evaluated for quantitative assay (Bethesda assay). Results: Out of total 276 patients, 243 patients of HA and 33 patients of HB were observed. The incidence of inhibitor development is 20.57% in HA and 6.06% in HB. The maximum number of patients and maximum number of inhibitors was between the age group of 11 and 30 years. There was more number of patients with severe disease as compared to mild and moderate forms. The concentration of inhibitor >5 BU was seen in 76% of HA patients and 100% of HB patients with inhibitor. Sixty-one patients came for follow-up. In three patients, inhibitor disappeared. The incidence of complications was more in patients who had developed inhibitor which increases the cost of treatment and increases the social suffering of the patients. Conclusion: Inhibitor development affect the severity and treatment of the disease significantly and there by increases the suffering and cost to the patient.