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