CLINICAL PROFILE, DISEASE SEVERITY, AND MANAGEMENT OF HEMOPHILIA PATIENTS: A CROSS-SECTIONAL STUDY FROM NORTH BENGAL

Background: Hemophilia is the most common inherited severe bleeding disorder, with limited data available from the resource-constrained regions of North Bengal. The establishment of the Hemoglobinopathies and Hemophilia Control Unit (HHCU) has facilitated structured diagnosis and management of hemophilia across India, including the sub-Himalayan region of North Bengal. The objective is to evaluate the clinicodemographic profile, disease severity, clinical manifestations, and management practices of hemophilia patients attending a tertiary care center in North Bengal. Materials and Methods: A hospital-based cross-sectional study was conducted at a tertiary care hospital from January 2022 to June 2026. All diagnosed hemophilia patients attending the HHCU during the study period were included. Clinical characteristics, family history, coagulation parameters (PT/INR and aPTT), factor assay findings, and treatment details were collected and analyzed. Results: A total of 76 patients with hemophilia were enrolled in the study. Among them, 52 (68.4%) patients belonged to the 11–25 years age group, while 24 (31.6%) were between 1 and 10 years of age. Of the 76 patients, 70 (92.1%) were diagnosed with Hemophilia A and 6 (7.9%) with Hemophilia B. Based on factor activity levels, 40 (52.6%) patients had severe disease, 31 (40.8%) had moderate disease, and 5 (6.6%) had mild disease. The most common clinical presentation was hemarthrosis, observed in 39 (51.3%) patients, followed by gum bleeding in 18 (23.7%), epistaxis in 10 (13.2%), and joint deformities in 9 (11.8%) patients. Prolonged activated partial thromboplastin time (aPTT) was observed in most patients, whereas prothrombin time/international normalized ratio (PT/INR) remained within the normal range. Recombinant factor concentrates were administered on demand to 58 patients, while prophylactic therapy was initiated in 5 children before the development of established joint deformities. All patients were provided with government-issued unique identification cards. Conclusion: Hemophilia A with severe factor deficiency was the predominant disease pattern in this cohort. The HHCU has substantially improved access to diagnosis, factor replacement therapy, prophylactic care, rehabilitation services, and patient awareness in North Bengal. Despite these advances, significant gaps remain in specialized diagnostic facilities and comprehensive long-term care, particularly for patients residing in rural areas.