Hemophilia A and B

Hemophilia

• Hemophilia A and B are X-linked transmitted disorders characterized by a lack of coagulation factor VIII (FVIII) or coagulation factor IX (FVIII), respectively, or by antibodies against FVIII or FIX.
• Although females may carry a trait, with very mild clinical signs, hemophilia A is mainly found in males.
• Severe hemophilia (factor activity <1%) is associated with severe spontaneous bleeding into major joints (elbows, knees and ankles) leading to hemarthroses, intramuscular bleeding, and retroperitoneal bleeding.
• Moderate hemophilia (factor activity 1% – 5%) is associated with less significant bleeding.
• Mild hemophilia (factor acitivity >5% – <40%) usually only manifests as impaired hemostasis after severe trauma or surgical procedures.
• Total joint replacements are not uncommon in patients with severe hemophilia.

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Related Pages

• Medical Disorders
  • Bleeding Disorders
• Oral Health Care Considerations
  • Bleeding Disorders
• Laboratory Values
  • Bleeding Disorders
  • Bleeding Tendencies

References/Websites

• References
  • Boender J, et al. A diagnostic approach to mild bleeding disorders. J Thromb Haemost. 2016 Aug;14(8):1507-16.
  • Halpern LR, et al. Treatment of the Dental Patient with Bleeding Dyscrasias: Etiologies and Management Options for Surgical Success in Practice. Dent Clin North Am. 2020;64(2):411-434.
  • Kizilocak H, et al. Diagnosis and treatment of hemophilia. Clin Adv Hematol Oncol. 2019 Jun;17(6):344-351.
  • Pelland-Marcotte M-C, et al. Hemophilia in a Changing Treatment Landscape. Hematol Oncol Clin North Am. 2019 Jun;33(3):409-423.
• Websites
  • Bleeding Disorders. MedlinePlus. U.S. National Library of Medicine. March 23, 2021.
  • What is Hemophilia? Centers for Disease Control and Prevention. July 17, 2020.