pattern is normal, whereas previously it was thought that angiodysplasia was associated with VWD subtypes lacking high-molecular weight multimers (HMWM). In an older, large international survey conducted in 4503 VWD… Click to show full abstract
pattern is normal, whereas previously it was thought that angiodysplasia was associated with VWD subtypes lacking high-molecular weight multimers (HMWM). In an older, large international survey conducted in 4503 VWD patients, angiodysplasia was reported only in patients with type 2 and type 3 VWD [3]. More recently, the VWD Prophylaxis Network reported angiodysplasia in nine patients with type 1 VWD [9], in addition to three other cases reported by a group from Italy [10]. Based on the antiangiogenic properties ascribed to VWF, it is possible that the level of functional VWF is also of importance as well as the loss of HMWM specifically. As both VWD and vascular malformations are relatively common, case–control studies are needed to elucidate whether VWF also has a role in the development of extra-intestinal vascular malformations. In future cases of VWD with extra-intestinal angiodysplasias, it would be worthwhile genotyping for endoglin and ACVRL1 mutations for possible coinheritance of HHT. Likewise, it would be interesting to conduct a study examining the prevalence of nasal angiodysplasias in VWD patients with refractory epistaxis. These efforts could elucidate whether what we are reporting is a coincidence or a related pathogenic process.
               
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