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Hemophilia

more about Hemophilia


  • Factor VIII deficiency (Factor 8 deficiency), or Hemophilia A, is the commonly called Hemophila.
  • Christmas disease (hemophilia B) (Factor 9 deficiency), has the same symptoms but is rarer.  Only about 15% of all Hemophila cases are Hemophilia B.

  • One of the ways that blood clots is through a cascade of factors.  Hemophilia is a bleeding disorder caused by a deficiency of blood clotting factors.
  • It is genetically inherited and almost exclusively affects males.
  • It can be divided into hemophilia A and hemophilia B (also called Christmas Disease); hemophilia A is much more common (accounting for 80% of all cases).

  • Bleeding in the joints: knees, ankles, or elbows.
  • Bleeding into the muscles
  • Bleeding from the rectum or vomiting blood
  • Severe hemophilia: less than 1% Factor VIII or Factor IX

- Spontaneous bleeding or bleeding with very minor Trauma

  • Moderate hemophilia: between 1% and 5% Factor VIII or Factor IX

- Bleeding with moderate injury

  • Mild hemophilia: more than 1% Factor VIII or Factor IX

- Bleeding only with significant Trauma


  • Lack of production of clotting factors
    1. Low levels of Factor VIII -- hemophilia A
    2. Low levels of Factor IX -- hemophilia B
    3. Low levels of Factor XI (Factor eleven) is sometimes called Hemophilia C.

  • Factor VIII and Factor IX assays: Decreased Factor VIII (hemophilia A) or Factor IX (hemophilia B)
  • There are mild, moderate, and severe forms of hemophilia A, depending the depletion levels of Factor VIII.
  • Prolonged activated partial thromboplastin time (APTT)
  • HIV testing -- a high number of persons with hemophilia developed HIV due to transfusions of missing factors which are blood products (occurred in the time before proper screening)

  • Replacement treatment
    1. Factor VIII concentrate transfusions (now heat-treated to reduce HIV transmission).  The goal level of Factor VIII depends on the severity of the bleeding problems being treated
    2. Factor IX concentrate transfusions for hemophilia B
  • Desmopressin acetate (DDAVP) -- for mild or moderate hemophilia A
  • Aminocaproic acid, Tranexamic acid for oral bleeding
  • Avoid aspirin and anti-inflammatory medications (e.g., ibuprofen)




more about Hemophilia


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