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Effectiveness of individualized management using WILFACTIN® in patients with von Willebrand disease during surgical procedures: A single-center study

      The symptomatic form of von Willebrand disease (VWD) affects 0.01 % of the general population. VWD is caused by quantitative defect (types 1 and 3) or qualitative defect (types 2A, 2B, 2M, and 2N) in von Willebrand factor (VWF), and the treatment to prevent bleeding during surgery goes from desmopressin to replacement therapy using different types of VWF concentrates containing variable levels of Factor VIII (FVIII) [
      • Laffan M.A.
      • Lester W.
      • O’Donnell J.S.
      • Will A.
      • Tait R.C.
      • Goodeve A.
      • et al.
      The diagnosis and management of von Willebrand disease: a United Kingdom Haemophilia Centre Doctors Organization guideline approved by the British Committee for Standards in Haematology.
      ]. Recent guidelines for the management of VWD have pointed that, because no comparative study is available, recommending standard algorithms for patients undergoing major or minor surgeries is difficult, and individualized therapy plans are necessary [
      • Connell N.T.
      • Flood V.H.
      • Brignardello-Petersen R.
      • Abdul-Kadir R.
      • Arapshian A.
      • Couper S.
      • et al.
      ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease.
      ]. There is a large range of dose and duration of treatment according to the type of VWF concentrates used in previous studies and moreover the target perioperative VWF and FVIII levels remain debated [
      • Goudemand J.
      • Bridey F.
      • Claeyssens S.
      • Itzhar-Baïkian N.
      • Harroche A.
      • Desprez D.
      • et al.
      Management of von Willebrand disease with a factor VIII-poor von Willebrand factor concentrate: results from a prospective observational post-marketing study.
      ,
      • Rugeri L.
      • d’Oiron R.
      • Harroche A.
      • Proulle V.
      • Mourey G.
      • De Raucourt E.
      • et al.
      Effectiveness and safety of hFVIII/VWF concentrate (Voncento®) in patients with inherited von Willebrand disease requiring surgical procedures: the OPALE multicentre observational study.
      ,
      • Srivastava A.
      • Serban M.
      • Werner S.
      • Schwartz B.A.
      • Kessler C.M.
      Wonders Study Investigators
      Efficacy and safety of a VWF/FVIII concentrate (wilate® ) in inherited von Willebrand disease patients undergoing surgical procedures.
      ]. We present herein the results of a single-center study that aimed to evaluate the modalities of treatment with a FVIII-poor plasma-derived VWF (pdVWF) concentrate: WILFACTIN® (LFB-Biomedicament, Les Ulis, France), during an individualized management of VWD patients who underwent surgery, depending on the type of VWD and the bleeding risk associated with each procedure.
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