Clinicopathological features of hemophilia in a tertiary care centre of India.

Context: Inherited bleeding disorders are common in India and hemophila and von Willebrand diseases are the most common among them. These patients can present in any department including paediatrics, medicine, orthopaedics and even gynaecology so knowledge about hemophilias and facilities for specialized tests for diagnosis are required. Few centres of north-eastern part of India perform these tests so hemophilias remain an underdiagnosed and underreported disease. Aims: The objective of this study was to estimate the prevalence of hemophilia in patients referred to this tertiary care centre and study the clinicopathological profile of these patients. Settings and Design: Prospective study. Methods and Material: Patients referred with suspicion of bleeding disorders in a time period of 4 years were evaluated. Complete clinical details, family history was retrieved and tests like complete blood counts, bleeding time, prothrombin time, activated partial thromboplastin time and factor assays were performed. Results: A total of 1126 patients with suspected bleeding disorder were tested and 237 were diagnosed of inherited bleeding disorders. Hemophilia A (HA) was diagnosed in 151 patients (63.7%), Hemophilia B (HB) in 31 (13%). Mean age was 10 years in HA and 11 years in HB patients. Clinical features of hemophilia varied according to Factor VIII levels. Coagulation type of bleeding such as hemarthrosis and hematoma were much more frequent than mucosal type bleeding. Conclusions: The present study is one of the very few studies from the north-eastern part of India estimating the prevalence and clinicopathological features of hemophilia, highlighting the need of specialized diagnostic facilities in this part of India. [ABSTRACT FROM AUTHOR]