title: Von Willebrand Factor Deficiency Corrected by Lung Transplantation.
abstract: BACKGROUND
  In experimental models with von Willebrand disease pigs, plasma von Willebrand factor (vWF) was significantly increased after lung transplantation because lung endothelial cells strongly express vWF. However, these findings have not been confirmed in human beings.
  
  
  METHODS
  A 26-year-old man with mild vWF deficiency (FvW:antigen 39 IU/dL; FvW:ristocetin cofactor activity 44 IU/dL; factor VIII 99%; normal multimeric plasma vWF pattern) was referred to our institution and underwent bilateral lung transplantation for cystic fibrosis. The patient received factor VIII/vWF concentrate both during and after surgery without any relevant bleeding events. At hospital discharge, he was taking immunosuppression with oral tacrolimus, prednisone, and mycophenolate mofetil, which has continued until the present day (22 months after the procedure).
  
  
  RESULTS
  Plasma vWF level increased during the postoperative days, presumably due to endothelial injuries and the infusion of vWF concentrate. Laboratory tests at 5, 11, 14, and 22 months after lung transplantation demonstrated sustained normalization of all parameters.
  
  
  CONCLUSIONS
  To our knowledge, this is the first reported case of von Willebrand deficiency corrected through lung transplantation.
authors.affiliation: 1 Department of Hematology, Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain. 2 Department of Thoracic Surgery, Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain. 3 Department of Pneumology, Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain. 4 Department of Hematology, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Rafael
authors.initials: R
authors.lastname: Forés
authors.forename: Ana
authors.initials: A
authors.lastname: Lario
authors.forename: Santiago
authors.initials: S
authors.lastname: Gil
authors.forename: Jose-Luis
authors.initials: JL
authors.lastname: Campo-Cañaveral
authors.forename: David
authors.initials: D
authors.lastname: Gomez-De-Antonio
authors.forename: Rosalia
authors.initials: R
authors.lastname: Laporta
authors.forename: Monica
authors.initials: M
authors.lastname: Martin
authors.forename: Grace
authors.initials: G
authors.lastname: Anze
authors.forename: Nieves
authors.initials: N
authors.lastname: Dorado
authors.forename: Jose-Luis
authors.initials: JL
authors.lastname: Bueno
authors.forename: Rafael
authors.initials: R
authors.lastname: Cabrera
mesh_terms: D000328:Adult; D006801:Humans; D016040:Lung Transplantation / Q000379:methods*; D008297:Male; D014842:von Willebrand Diseases / Q000097:blood / Q000601:surgery*; D014841:von Willebrand Factor / Q000378:metabolism*
chemical_list: D014841:von Willebrand Factor