Hemophilia and von Willebrand Disease: A Review of Emergency Department Management



      Hemophilia and von Willebrand disease (VWD) are the most common congenital coagulation factor deficiencies. Patients with these disorders who experience bleeding complications are often initially managed in the emergency department (ED).

      Objective of the Review

      This review will focus on the emergency department management of patients with these disorders and provide an update on current treatment options.


      The mainstay of management is initial stabilization, control of bleeding when possible, and administration of specific factors. Early coordination of care with hematology is critical.


      Emergency medicine providers must have an understanding of the pathophysiology, clinical presentation, and management strategies in order to optimally care for these complex patients.


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        • Singleton T.
        • Kruse-Jarres R.
        • Leissinger C.
        Emergency department care for patients with hemophilia and von Willebrand disease.
        J Emerg Med. 2010; 39: 158-165
        • Srivastava A.
        • Brewer A.K.
        • Mauser-Bunschoten E.P.
        • et al.
        Guidelines for the management of hemophilia.
        Haemophilia. 2013; 19: e1-e47
        • Antonarakis S.E.
        Molecular genetics of coagulation factor VIII gene and hemophilia A.
        Thromb Haemost. 1995; 74: 322-328
        • Blanchett V.S.
        • Key N.S.
        • Ljung L.R.
        • et al.
        Definitions in hemophilia: communication from the SSC of the ISTH.
        J Thromb Haemost. 2014; 12: 1935-1939
        • Sabatine M.
        • Morrow D.
        • Giugliano R.
        • et al.
        Association of hemoglobin levels with clinical outcomes in acute coronary syndromes.
        Circulation. 2005; 111: 2042-2049
        • Berntorp E.
        • Halimeh S.
        • Gringeri A.
        • Mathias M.
        • Escuriola C.
        • Pérez R.
        Management of bleeding disorders in children.
        Haemophilia. 2012; 18: 15-23
        • Hanley J.
        • McKernan A.
        • Creagh M.D.
        • et al.
        Guidelines for the management of acute joint bleeds and chronic synovitis in haemophilia: A United Kingdom Haemophilia Centre Doctor’s Organization (UKHCDO) guideline.
        Hemophilia. 2017; 23: 511-520
        • Ashrani A.A.
        • Osip J.
        • Christie B.
        • Key N.S.
        Iliopsoas haemorrhage in patients with bleeding disorders--experience from one centre.
        Haemophilia. 2003; 9: 721-726
        • Balkan C.
        • Kavakli K.
        • Karapinar D.
        Iliopsoas haemorrhage in patients with haemophilia: results from one centre.
        Haemophilia. 2005; 11: 463-467
        • Zimmerman B.
        • Valentino L.A.
        Hemophilia: in review.
        Pediatr Rev. 2013; 34: 289-295
        • Witmer C.M.
        • Rafni L.J.
        • Manno C.S.
        Utility of computed tomography of the head following head trauma in boys with haemophilia.
        Haemophilia. 2007; 13: 560-566
        • Antunes S.V.
        • Vicari P.
        • Cavalheiro S.
        • Bordin J.O.
        Intracranial haemorrhage among a population of haemophilic patients in Brazil.
        Haemophilia. 2003; 9: 573-577
        • Sorensen B.
        • Benson G.M.
        • Bladen M.
        • et al.
        Management of muscle haematomas in patients with severe haemophilia in an evidence poor world.
        Haemophilia. 2012; 18: 598-606
        • Melchiorre D.
        • Linari S.
        • Innocenti M.
        • et al.
        Ultrasound detects joint damage and bleeding in haemophilic arthropathy: a proposal of a score.
        Haemophilia. 2011; 17: 112-117
        • National Hemophilia Foundation Medical and Scientific Advisory Council (MASAC)
        Guidelines for emergency department management of individuals with hemophilia and other bleeding disorders. MASAC Document 252.
        (Available at:)
        • National Hemophilia Foundation Medical and Scientific Advisory Council (MASAC)
        MASAC recommendations concerning products licensed for the treatment of hemophilia and other bleeding disorders.
        MASAC Document. 2017; 250 (Available at:)
        • Cuifir L.
        • Kempton C.
        Current and emerging factor VIII replacement products for hemophilia A.
        Ther Adv Hemotol. 2017; 8: 303-313
        • Droubatchevskaia N.
        • Wong M.
        • Chipperfield K.
        • Wadsworth L.
        • Fergusson D.
        Guidelines for cryoprecipitate transfusion.
        BC Med J. 2007; 49: 441-445
        • Revel-Vilk S.
        • Blanchette V.S.
        • Sparling C.
        • Stain A.M.
        • Carcao M.D.
        DDAVP challenge tests in boys with mild/moderate haemophilia A.
        Br J Haematol. 2001; 117: 947-951
        • Gill J.C.
        • Ottum M.
        • Schwartz B.
        Evaluation of high concentration intranasal and intravenous desmopressin in pediatric patients with mild hemophilia A or mild-to-moderate type 1 von Willebrand disease.
        J Pediatr. 2002; 140: 595-599
        • Shander A.
        • Walsch C.
        • Bailey H.
        • Cromwell C.
        Acquired hemophilia presenting as profound hematuria: evaluation, diagnosis, and management of elusive cause of bleeding in the emergency department setting.
        J Emerg Med. 2013; 45: e1-e6
        • Kruse-Jarres R.
        • Kempton C.
        • Baudo F.
        • et al.
        Acquired hemophilia A: updated review of evidence and treatment guidance.
        Am J Hematol. 2017; 92: 695-705
        • Huth-Kuhne A.
        • Baudo F.
        • Collins P.
        • et al.
        International recommendations on the diagnosis and treatment of patients with acquired hemophilia A.
        Haematologica. 2009; 94: 566-575
        • Rodeghiero F.
        • Castaman G.
        • Dini E.
        Epidemiological investigation of the prevalence of Von Willebrand disease.
        Blood. 1987; 69: 454-459
        • Nichols W.L.
        • Hultin M.B.
        • James A.H.
        Von Willebrand disease: evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute Expert Panel report (USA).
        Haemophilia. 2008; 14: 171-232
        • Veyradier A.
        • Boisseau P.
        • Fressinaud E.
        • Caron C.
        • Ternisien C.
        A laboratory phenotype/genotype correlation of 1167 French patients from 670 families with von Willebrand disease: a new epidemiologic picture.
        Medicine (Baltimore). 2016; 95: e3038
        • Peyvandi F.
        • Garagiola I.
        • Baronciani L.
        Role of von Willebrand factor in the haemostasis.
        Blood Transfus. 2011; 9: s3-s8
        • Swami A.
        • von Willebrand disease K.V.
        a concise review and update for the practicing physician.
        Clin Appl Thromb Hemost. 2017; 23: 900-910
        • Sander Y.V.
        • Fijnvandraat K.
        • Boender J.
        Bleeding spectrum in children with moderate or severe von Willebrand disease: relevance of pediatric-specific bleeding.
        Am J Hematol. 2015; 9: 1142-1148
        • Leebeek F.W.
        • Eikenboon J.C.
        Von Willebrand’s disease.
        N Engl J Med. 2016; 375: 2057-2080
        • van Galen K.P.M.
        • Mauser-Bunschoten E.P.
        • Leebeek F.W.G.
        Hemophilic arthropathy in pateints with von Willebrand disease.
        Blood Rev. 2012; 26: 261-266
        • Michiels J.J.
        • van de Velde A.
        • van Vliet H.H.
        • van der Planken M.
        • Schroyens W.
        • Berneman Z.
        Response of von Willebrand factor parameters to desmopressin in patients with type 1 and type 2 congenital von Willebrand disease: diagnostic and therapeutic implications.
        Semin Thromb Hemost. 2002; 28: 111-132
        • Federici A.B.
        • Mazurier C.
        • Berntorp E.
        • et al.
        Biologic response to desmopressin in patients with severe type 1 and type 2 von Willebrand disease: results of a multicenter European study.
        Blood. 2004; 103: 2032-2038
        • Siew D.A.
        • Mangel J.
        • Laudenbach L.
        • Schembri S.
        • Minuk L.
        Desmopressin responsiveness at a capped dose of 15 μg in type 1 von Willebrand disease and mild hemophilia A.
        Blood Coagul Fibrinolysis. 2014; 25: 820-823
        • Leissinger C.
        • Carcao M.
        • Gill J.C.
        • Journeycake J.
        • Singleton T.
        • Valentino L.
        Desmopressin (DDAVP) in the management of patients with congenital bleeding disorders.
        Haemophilia. 2014; 20: 158-167
        • Mannucci P.
        • Bettega D.
        • Cattaneo M.
        Patterns of development of tachyphylaxis in patients with haemophilia and von Willebrand disease after repeated doses of desmopressin (DDAVP).
        Br J Haematol. 1992; 82: 87-93
        • Vicente V.
        • Estellés A.
        • Laso J.
        • Moraleda J.
        • Rivera J.
        • Aznar J.
        Repeated infusions of DDAVP induce low response of FVIII and vWF but not of plasminogen activators.
        Thromb Res. 1993; 70: 117-122
        • Achinger S.
        • Arieff A.
        • Kalantar-Zadeh K.
        • Ayus J.
        Desmopressin acetate (DDAVP)-associated hyponatremia and brain damage: a case series.
        Nephrol Dial Transplant. 2014; 29: 2310-2315
        • Stoof S.C.M.
        • Cnossen M.H.
        • de Maat M.P.M.
        • Leebeek F.W.G.
        • Kruip M.J.H.A.
        Side effects of desmopressin in patients with bleeding disorders.
        Haemophilia. 2016; 22: 39-45
        • National Hemophilia Foundation Medical and Scientific Advisory Council (MASAC)
        MASAC recommendations concerning products licensed for the treatment of hemophilia and other bleeding disorders.
        2018 (MASAC Document 253. Available at:)
        • Singal M.
        • Kouides P.
        Recombinant von Willebrand factor: a first-of-its-kind product for von Willebrand disease.
        Drugs Today (Barc). 2016; 52: 653-664
        • Peyvandi F.
        • Kouides P.
        • Turecek P.
        • Dow E.
        • Berntorp E.
        Evolution of replacement therapy for von Willebrand disease: from plasma fraction to recombinant von Willebrand factor.
        Blood Rev. 2019; 38: 100572
        • Lavin M.
        • O’Donnell J.
        New treatment approaches to von Willebrand disease.
        Hematology Am Soc Hematol Educ Program. 2016; 2016: 683-689
        • Eghbali A.
        • Melikof L.
        • Taherahmadi H.
        • Bagheri B.
        Efficacy of tranexamic acid for the prevention of bleeding in patients with von Willebrand disease and Glanzmann thrombasthenia: a controlled, before and after trial.
        Haemophilia. 2016; 22: e423-e426
        • Kouides P.A.
        • Byams V.R.
        • Philipp C.S.
        • et al.
        Multisite management study of menorrhagia with abnormal laboratory haemostasis: a prospective crossover study of intranasal desmopressin and oral tranexamic acid.
        Br J Haematol. 2009; 145: 212-220
        • van Galen K.P.
        • Engelen E.T.
        • Mauser-Bunschoten E.P.
        • van Es R.J.
        • Schutgens R.E.
        Antifibrinolytic therapy for preventing oral bleeding in patients with haemophilia or von Willebrand disease undergoing minor oral surgery or dental extractions.
        Cochrane Database Syst Rev. 2015; 12: CD011385

      Linked Article

      • Emergency Management of Hemophilia
        Journal of Emergency MedicineVol. 59Issue 4
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          We read with interest the review article by Tebo et al., which we hope will help raise awareness of hemophilia and von Willebrand disease among emergency physicians (1). We would like to emphasize and clarify some aspects of the urgent assessment and management of such patients in the emergency department.
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