Abstract Number: PB1592
Meeting: ISTH 2020 Congress
Background: Von Willebrand’s disease (vWD) is the most common congenital bleeding disorder. The revised 2014 British Journal of Haematology (BJH) guidelines provide a new framework by which to classify vWD subtypes. This includes a new category for patients with a milder reduction in vWF level who are now classified as ‘low vWF’.
Aims: To perform a diagnostic evaluation of patients with a vWD diagnosis using the 2014 BJH guidelines and to correlate their revised diagnosis with bleeding patterns and management.
Methods: A retrospective observational study involving analysis of 227 vWD patients at Addenbrooke’s Hospital. Each diagnosis was reclassified according to the 2014 BJH guidelines. Further data was collected from electronic patient records including symptoms and management.
Results: Based on the 2004 BJH guidelines, 70% of patients were diagnosed with type 1, 29% with type 2 and 1% with type 3 vWD.
After reclassification, the proportion of patients diagnosed with types 2 and 3 vWD remained largely unchanged at 28% and 1% respectively. However, of the 70% of patients originally diagnosed with type 1 vWD, 16% retained this subtype. The remainder were reclassified as ‘low vWF’ (42%) or with a normal vWF level (13%).
The pattern of bleeding symptoms for patients with types 1 and 2 vWD were similar. In those with type 1, the proportion with bleeding symptoms reduced compared to those with type 2 after reclassification.
Desmopressin and tranexamic acid were commonly used treatments across all subtypes. Notably, vWF concentrate was increasingly used for more severe phenotypes (types 2 and 3). This remained unchanged following reclassification.
Conclusions: In our cohort, fewer patients were diagnosed with vWD after reclassification. A large proportion of patients are now classified with ‘low vWF’. These stricter criteria allow a greater diagnostic accuracy, thus avoiding unnecessary diagnosis in those with a milder reduction in vWF.
To cite this abstract in AMA style:Bar E, De Soysa S, Huang L, Symington E. Von Willebrand’s Disease: A Diagnostic Evaluation Using Current Guidelines [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/von-willebrands-disease-a-diagnostic-evaluation-using-current-guidelines/. Accessed December 5, 2021.
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