Article Abstract

Von Willebrand disease in Iran: diagnosis and management

Authors: Akbar Dorgalaleh, Shadi Tabibian, Mahmood Shams, Fereydoun Ala, Gholamreza Bahoush, Mohammad Jazebi, Rima Manafi, Behnaz Tavasoli, Mohammad Reza Baghaipour


von Willebrand disease (VWD) is the most common inherited bleeding disorder, and is classified into quantitative (type 1 and type 3) and qualitative (type 2) defects. The bleeding tendency is variable among affected patients, ranging from an asymptomatic condition, mostly in type 1, to severe life-threatening diathesis, mostly in type 3. Type 3 VWD is inherited in an autosomal recessive manner and therefore is more frequent in areas with high rate of consanguinity such as Iran. As in many other parts of the world, the precise prevalence of VWD is not clear in Iran, but the incidence of type 3 VWD is higher than is usual in other countries. The diagnosis of VWD is challenging in Iran, with limited diagnostic tests routinely available, especially compromising the precise diagnosis and classification of type 2 VWD. Patients with VWD in Iran present variable clinical manifestations, with epistaxis and menorrhagia representing the most common mucosal hemorrhages. Different therapeutic choices are available for management of these patients in Iran including cryoprecipitate as a traditional choice, and desmopressin, factor VIII/ von Willebrand factor concentrate (Humate-P and Wilate) and adjuvant agents representing more modern and effective alternatives.