Etiology, Clinical Profile, and Demographics of Hemophilia B in Eastern India

Background : The most common severe inherited hemorrhagic disease is hemophilia, which means "love of blood." Deficiencies in Factors VIII and IX cause hemophilia, with inadequate levels or malfunctioning of these factors being the primary causes of hemophilia A and B, respectively. This study aims to assess the incidence and characteristics of inherited coagulation disorders, particularly hemophilia B. Objective: The present investigation aims to evaluate the incidence and clinical traits of congenital anticoagulation diseases, specifically hemophilia B. Method : The study included 98 Hemophilia B patients with a mean age of 25. Medical histories were recorded using a predesigned Performa, covering age, gender, bleeding site, and family history. Result: The study included 98 hemophilia B patients, with 39 having severe and 59 moderate forms. The majority (60.2%) had moderate hemophilia, while 39.7% had the severe type. Most patients (17.4 ± 10.5 years) were aged 21-30, with the mean age of onset being 17.4±10.5 years for severe hemophilia and 8.6±4.43 years for moderate cases. Knee joints were the most affected, found in 79.5% of cases and accounting for 98.9% of target joint infections, followed by elbow joints at 90.8%. Regular factor IX replacement was needed in 39.7% of severe cases. This highlights the importance of early diagnosis and treatment for managing joint-related complications in hemophilia B patients. Conclusion: The current study examines hemophilia B's sociodemographic and clinic-pathological characteristics. Early diagnosis may be beneficial only when the spectral manifestation of hemophilia B in the community is known.