PB0233 - Von Willebrand disease in the east Mediterranean region: current situation and gaps

Background: According to of the Word Federation of Hemophilia annual survey, von Willebrand disease is underrepresented in the eastern Mediterranean region with a maximum prevalence of 0.27/100000

Aims: To understand the gaps and improve the current situation within the east Mediterranean region The HEMNET group build a survey to collect information concerning Von Willebrand disease within the region

Methods: A google survey form was sent to 250 hematologists identified by the HEMNET group and working in the 20 countries of the region.

Results: 74 responses were obtained from 16 countries
Bleeding score was a tool of diagnosis for 61,1% and pictorial assessment chart for 23.6%
98% of respondents state that complete blood count, APTT, FVIII activity and Von Willebrand antigen were available, while 87%, 16% and 41% respectively state that VWF Rco, VWF: CB and RIPA were available. Genetics and multimers studies were available for less than 9%
Tranexamic acid, Desmopressin and Von Willebrand concentrate are respectively available for 94.6%, 54.5%, 83.8% of respondents. They used them in 90.5%, 45.2%, 89.2% respectively. Nevertheless, cryoprecipitate and fresh frozen plasma are still used for 50% of respondent.
Pharmacokinetic was never proposed for 75% of respondent and 15% did not proposed DDAVP test while available
For menorrhagia first line treatment was tranexamic acid for 79.7%, factor concentrate for 32.4% and hormonal therapies for 47.3%
59.4% have experience in surgery. Modalities assessment during surgery were clinical assessment for 39.4%. VWF AG and VWF Rco were performed for 33.6% and 26.6% respectively. Laboratory assessment was obtained on time only for 22.4%.
62.3% do not have registry for Willebrand disease and only 23% have outreach program.
The New Von Willebrand Guideline was considered not applicable for 5.7% while 28.6% did not know about it .

Conclusion(s): Improved awareness, simplified diagnostic approach and easy access to treatment are needed