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
Asian Journal of Medical Principles and Clinical Practice,
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.
- Von Willebrand disease
- BAT score
- family history
- bleeder screening methods
How to Cite
Hoffbrand A, Higgs DR, Keeling DM, Mehta AB. Postgraduate Haematology: Seventh Edition; 2015.
Khan MK, Khan SQ, Malik NA. Spectrum of Von Willebrand disease in Punjab: Clinical Features and Types. 2014; 26(4):470–3.
Tosetto A, Castaman G, Rodeghiero F. Evidence-based diagnosis of type 1 von Willebrand disease: a Bayes theorem approach. 2008;111(8):3998–4003.
Favaloro EJ, Soltani S, McDonald J, Grezchnik E, Easton L, Favaloro JWC. Reassessment of ABO Blood Group, Sex, and Age on Laboratory Parameters Used to Diagnose von Willebrand Disorder. Am J Clin Pathol. 2005;124(6):910–7.
Goodeve A, Goodeve AC. The genetic basis of von Willebrand disease The genetic basis of von Willebrand disease; 2016 (March).
Fuchs B. Structure-function relationship of von Willebrand factor; 2009 (February).
Goodeve JC, Giancarlo R, Francesco F, Augusto BB, Javier M, Dominique M, et al, Phenotype and genotype of a cohort of families historically diagnosed with type 1 von Willebrand disease in the European study, Molecular and Clinical Markers for the Diagnosis and Management of Type 1 von Willebrand Disease (MCMDM-1VWD). Blood. 2006; 109(1):112–21.
James PD, Lillicrap D. von Willebrand disease: Clinical and laboratory lessons learned from the large von Willebrand disease studies. Am J Hematol. 2012;87(SUPPL. 1):4–11.
Trasi S, Shetty S, Ghosh K, Mohanty D. Prevalence & spectrum of von Willebrand disease from western India. Indian Journal of Medical Research. 2005;121(5):653–658.
Roberts JC, Flood VH. Laboratory diagnosis of von Willebrand disease. International Journal of Laboratory Hematology. 2015;37:7–11.
Laffan MA, Lester W, O’Donnell JS, Will A, Tait RC, Goodeve A, Millar CM, et al, The diagnosis and management of von Willebrand disease: A United Kingdom Haemophilia Centre Doctors Organization guideline approved by the British Committee for Standards in Haematology.
Khan MK, Aleem Khan, Asuria SQ, Malik NA Slam. Spectrum of Von Willebrand’s disease in Punjab: clinical features and types. Journal of Ayub Medical College, Abbottabad: JAMC. 2014;26(4): 470–473.
Castaman G, Montgomery RR, Meschengieser SS, Haberichter SL, Woods a I, Lazzari M a. von Willebrand’s disease diagnosis and laboratory issues. Haemophilia. 2010;16 Suppl 5:67–73.
World Federation of Hemophilia Report on the. World. Internet] pdf-1439.pdf (wfh.org); 2011.
Sap F, Kavaklı T, Kavaklı K, Dizdarer C. The Prevalence of von Willebrand Disease and Significance of in Vitro Bleeding Time (PFA-100) in von Willebrand Disease Screening in the İzmir Region. Turkish journal of haematology: official journal of Turkish Society of Haematology. 2013; 30(1):40–47.
Lewis SM, Bain BJ, Bates I. Dacie and Lewis Practical Haematology. 10th Ed Churchill Livingston Elsevier. 2006; 722.
Prevalence and spectrum of von Willebrand disease in Eastern Uttar Pradesh Kumar Sandip, Kishore Ruchi, Gupta Vineeta, Jain Madhu, Shukla Jyoti Indian Journal of Pathology and Microbiology. 2010;53(3):486-489.
Srivastava Alok, Rodeghiero Francesco. Epidemiology of von Willebrand Disease in Developing Countries. Seminars in thrombosis and hemostasis. 2005;31 :569-76.
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