Management of gastrointestinal bleeding in a patient with type 3 von Willebrand disease and inhibitors to von Willebrand factor: a case report

von Willebrand disease (VWD) is a rare bleeding disorder caused by deficiency of von Willebrand factor (VWF). Development of inhibiting alloantibodies to VWF in VWD after exposure to factor concentrates is very rare. We present a 56-year-old male patient with type 3 VWD and history of inhibitors to VWF who was hospitalized due to severe bleeding from the rectal angiodysplasia, which happened 6 months after radical irradiation therapy of the prostate adenocarcinoma. He was treated locally with argon plasma coagulation and application of peptide hydrogel, which resulted in progression of lesions to rectal ulcer covering two-thirds of the rectal mucosa. In addition to almost daily transfusions of red blood cells, he received recombinant activated factor VIIa (rFVIIa), tranexamic acid, FXIII concentrate and activated prothrombin complex concentrate (APCC). Infusion of VWF/FVIII concentrate resulted in reappearance of FVIII inhibitors. Ultimately, the bleeding stopped after introduction of off-label emicizumab, platelet transfusions and local application of the platelet-rich fibrin (PRF).