Mitrovic, Mirjana (54972086700)Mirjana (54972086700)MitrovicElezovic, Ivo (12782840600)Ivo (12782840600)ElezovicMiljic, Predrag (6604038486)Predrag (6604038486)MiljicSuvajdzic, Nada (7003417452)Nada (7003417452)Suvajdzic2025-07-022025-07-022014https://doi.org/10.1016/j.bcmd.2013.11.001https://www.scopus.com/inward/record.uri?eid=2-s2.0-84895539616&doi=10.1016%2fj.bcmd.2013.11.001&partnerID=40&md5=5d8245e4c659304c57c4e255452ad2echttps://remedy.med.bg.ac.rs/handle/123456789/13712Although various coagulation abnormalities occur in patients with Gaucher disease (GD), von Willebrand factor (vWF) deficiency has rarely been reported. A retrospective review of six treatment naïve cases with GD and concomitant vWF deficiency over a 12-year-period in a single center is presented. All patients had a personal history of prior hemorrhages. Based on both reduced level of vWF antigen (vWF:Ag, range 14-56%) and ristocetin cofactor activity (vWF:RCo, range 12-53%), with a vWF:RCo/Ag ratio >0.7, the diagnosis of type 1 von Willebrand disease was made in all six cases. During enzyme replacement therapy (ERT) of a 2-year duration all patients normalized their vWF:Ag levels. Based on the positive ERT effect on vWF:Ag levels, vWF deficiency was assumed to be acquired. It should be noted that beside vWF deficiency four patients with GD exhibited mild thrombocytopenia (range 81-131×109/L) and three had additional hemostatic defects (reduced collagen platelet aggregation, FV, FXI and FXII deficiencies). © 2013 Elsevier Inc.Acquired von Willebrand syndromeBleedingEnzyme replacement therapyGaucher disease