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Factor 8 deficiency
(Hemophilia A) or Factor 9 deficiency (Hemophilia B).
Hemophilia B is
also called,
Christmas disease
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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 5% of the normal amount of
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
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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 on how low the levels of Factor VIII are.
- 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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