Analysis of Clinical and Laboratory Characteristics in Patients with Von Willebrand Disease in a Five Year Follow Up at a Tertiary Referral Centre in Sri Lanka

Abstract

Background: Von Willebrand disease (vWD) is the commonest, inherited bleeding disorder worldwide. A retrospective cross-sectional study was done to analyze clinical and laboratory characteristics in 47 diagnosed patients with vWD and to assess the utility in basic bleeder screen and BAT (Bleeding Assessment Tool) score in a five year (2012-2017) follow up at a tertiary referral centre in Sri Lanka. Methods: Clinical assessment was done by using the BAT score. Initial screening tests of coagulation profile and bleeding time was analyzed. Ristocetin Induced Platelet Aggregation (RIPA), vonWillebrand-antigen, activity and Factor-VIII were also done to confirm and categorize the subtype of the disease. Results: The commonest type was type 1 (31, 70.2%) followed by type 2B/Platelet type (8, 17%) and type 3 (8, 17%). Type 3 disease showed the highest average BAT score followed by type 2B disease which is compatible with their bleeding severity. Bleeding time was prolonged in 21(44.7%) and was normal in 26 (55.3%) of patients. APTT was prolonged in only31(66%) and was normal in 16 (34%). A positive family history was seen in 27 (57.4%), negative family history in 17 (36.2%) patients and family history was unknown in 3(6.4%) patients. Type 3 and Type 1 VWD patients showed a higher association with a positive family history. Conclusion: In the diagnosis of vWD, the basic bleeder screening tests alone are not enough and using BAT score incorporated with family history is shown to be more useful. Therefore, combining both these will provide a better bleeder screening method for the vWD.