Review of Hemophilia A and B and Perioperative Management in Dentistry

  • Pooja Gangwani
  • Ryan Richards


Hemophilia A (classic hemophilia) and hemophilia B (Christmas disease) are inherited disorders of coagulation that can produce life-threatening bleeding. Patients with hemophilia will require special management strategies in order to safely undergo dental treatment. Patient management is optimized when the dental professional has a basic understanding of how these diseases affect hemostasis, is familiar with medical/systemic treatment strategies available to the hematologist, and knows the principles of dental/local management of patients with bleeding risks. In order to help with these goals, this chapter reviews the pathophysiology, clinical presentation, patient evaluation, and basics of medical management of hemophilia. These reviews are presented with an emphasis given to the dental management of patients with hemophilia A and B.


Hemophilia Hemophilia A Classic hemophilia Hemophilia B Christmas disease Inherited bleeding disorder Desmopressin Fresh frozen plasma Cryoprecipitate Coagulation factors Factors VIII Factor IX Epsilon-aminocaproic acid Tranexamic acid Tranfusion 


  1. 1.
    Zimmerman B, Valentino LA. Hemophilia: in review. Pediatr Rev. 2013;34:289–95.CrossRefGoogle Scholar
  2. 2.
    Smith JA. Hemophilia: what the oral and maxillofacial surgeon needs to know. Oral Maxillofac Surg Clin North Am. 2016;28:481–9.CrossRefGoogle Scholar
  3. 3.
    Srivastava A, Brewer AK, Mauser-Bunschoten EP, Key NS, Kitchen S, Llinas A, Ludlam CA, Mahlangu JN, Mulder K, Poon MC, Street A. Guidelines for the management of hemophilia. Haemophilia. 2013;19:1–47.CrossRefGoogle Scholar
  4. 4.
    Mensah PK, Gooding R. Surgery in patients with inherited bleeding disorders. Anesthesia. 2015;70:112–20.CrossRefGoogle Scholar
  5. 5.
    Kreuz W, Ettingshausen CE. Inhibitors in patients with haemophilia A. Thromb Res. 2014;134:S22–6.CrossRefGoogle Scholar
  6. 6.
    Ljung RCR. Intracranial hemorrhage in hemophilia A and B. Br J Haematol. 2007;140:378–84.CrossRefGoogle Scholar
  7. 7.
    Balkan C, Kavakli K, Karapinar D. Iliopsoas haemorrhage in patients with haemophilia: results from one centre. Haemophilia. 2005;11:463–7.CrossRefGoogle Scholar
  8. 8.
    Ljung R, Andersson NG. The current status of prophylactic replacement therapy in children and adults with hemophilia. Br J Haematol. 2015;169:777–86.CrossRefGoogle Scholar
  9. 9.
    Rodriguez-Merchan EC. Musculoskeletal complications of hemophilia. HSSJ. 2010;6:37–42.CrossRefGoogle Scholar
  10. 10.
    Franchini M, Zaffanello M, Lippi G. The use of desmopressin in mild hemophilia A. Blood Coagul Fibrinolysis. 2010;21:615–9.CrossRefGoogle Scholar
  11. 11.
    Haberichter SL, Shi Q, Montgomery RR. Regulated release of VWF and FVIII and the biologic implications. Pediatr Blood Cancer. 2006;46:547–53.CrossRefGoogle Scholar
  12. 12.
    Little, James W. Dental management of the medically compromised patient, 7th edn. St. Louis, MO; Mosby, 2008.Google Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Pooja Gangwani
    • 1
  • Ryan Richards
    • 1
  1. 1.Oral and Maxillofacial SurgerySt. Joseph’s University Medical CenterPatersonUSA

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