Prevalence and spectrum of von Willebrand disease in Eastern Uttar Pradesh

Abstract

Von Willebrand disease is the most common inherited bleeding disorder with a prevalence of approximately 1% in the general population. Studies available from India are limited, showing a prevalence of approximately 10% of vWD amongst inherited bleeding disorders. This study aims to know the prevalence and subtypes of vWD in patients presenting with various bleeding manifestations to university hospital. We investigated 840 patients presenting with bleeding manifestations in the period from August 2004 to August 2008 for bleeding disorders. Tests performed for the diagnosis of vWD included platelet count, bleeding time (BT), prothrombin time (PT), activated partial thromboplastin time (APTT), Factor VIII:C assay, von Willebrand Factor Antigen assay and Ristocetin-induced platelet aggregation. Amongst 840 patients, 230 (27.38%) had inherited bleeding disorder. Out of these, 40 (17.39%) patients were identified as vWD. Type 1 in 17 (42.5%), Type 2 in 11 (27.5%) and Type 3 vWD was present in 12 (30.0%) patients. Patients' age ranged from five months to 45 years, with 17 males and 23 females. Positive family history was seen in 12 patients (30%). Muco-cutaneous bleeding was the most common presentation. Menorrhagia was present in 100% women of reproductive age group, and hemarthrosis was seen in two male patients. We felt that Type I vWD with infrequent and mild bleeding episodes remain undiagnosed either because of unawareness of the disease in society or due to paucity of diagnostic facilities available in our country. Therefore, an awareness program along with enhancement of diagnostic facilities for vWD is required in our country to identify these patients for proper management thus avoiding hemorrhagic complications.