title: The use of desmopressin in congenital factor XI deficiency: a systematic review.
abstract: Factor XI (FXI) deficiency is a rare inherited coagulation disorder characterized by infrequent spontaneous bleeding, but increased risk of hemorrhagic complications especially after trauma or surgery. Treatment options for FXI-deficient patients include virus-inactivated fresh frozen plasma, plasma-derived FXI concentrates, and activated recombinant FVII. Inhibitors of fibrinolysis, such as tranexamic acid, and desmopressin (DDAVP) have also been used in these patients, especially in mild cases. The current knowledge on the use of the latter agent in this congenital bleeding condition is systematically reviewed here. Although limited, the available literature data suggest the potential role of DDAVP for either treatment of bleeding episodes or the prevention of postoperative bleeding in patients with milder FXI defects. However, these findings need to be supported by further trials on large population of patients.
authors.affiliation: Servizio di Immunoematologia e Medicina Trasfusionale, Dipartimento di Patologia e Medicina di Laboratorio, Azienda Ospedaliero-Universitaria di Parma, Parma, Italy. massimo.franchini@azosp.vr.it
authors.forename: Massimo
authors.initials: M
authors.lastname: Franchini
authors.forename: Franco
authors.initials: F
authors.lastname: Manzato
authors.forename: Gian Luca
authors.initials: GL
authors.lastname: Salvagno
authors.forename: Martina
authors.initials: M
authors.lastname: Montagnana
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Lippi
mesh_terms: D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use*; D005173:Factor XI Deficiency / Q000188:drug therapy*; D006470:Hemorrhage / Q000517:prevention & control; D006490:Hemostatics / Q000627:therapeutic use; D006801:Humans; D011183:Postoperative Complications
chemical_list: D006490:Hemostatics; D003894:Deamino Arginine Vasopressin