Hemophilia A natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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Overview

• Hemophilia A presentation varies depending on the stage of the disease:
  • People with mild hemophilia (5-40% of factor VIII activity in the blood) generally present with excessive bleeding following surgery (such as a dental procedure) or trauma. They may remain asymptomatic otherwise for long period of time, even into late adulthood
  • People with moderate hemophilia (1-5% of factor VIII activity in the blood) have presentation ranging between mild and severe forms. They present earlier than patients with mild hemophilia, and may bleed following minor trauma
  • People with severe hemophilia (less than 1% of factor VIII in blood) present sooner in life with abnormal bleeding episodes, usually in the first year of life. They are also at risk for spontaneous hemorrhages, i.e. unprovoked bleeding episodes, frequently in the joints and muscles

Natural History

• Clinical features are usually related to abnormal or spontaneous bleeding, and can be separated into internal bleeds and external bleeds
  • Internal bleeding sites include:
    • Hematuria (bleeding from kidneys or bladder)
    • Melena or hematochezia (bleeding into the GI tract)
    • Hemarthroses and hematomas (bleeding into joints and muscle)
    • Bleeding into the brain (potentially catastrophic)
  • External bleeding sites include:
    • Heavy bleeding from a minor cut
    • Excessive bleeding from surgical or traumatic wound
    • Epistaxis or bleeding in gums (though not as commonly seen as in platelet disorders)

Complications

• Many of the long-term sequelae of hemophilia A are either related to the morbidity of severe bleeds, or from side effects of frequent transfusions
  • Recurrent episodes of hemarthroses can cause arthritis and eventual destruction of the joint space
  • Frequent transfusions have resulted in high profile cases of hemophiliacs becoming infected with blood-borne viruses (i.e. HIV and Hepatitis C). With improved screening of blood products, the risks of these infections has been substantially reduced