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Christmas Disease

more about Christmas Disease

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 trauma

  • Mild Hemophilia: more than 5% Factor VIII or Factor IX

- Bleeding only with significant trauma

  • Lack of production of bleeding factors
  • Low levels of Factor VIII -- Hemophilia A
  • Low levels of Factor IX -- Hemophilia B

  • 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)

  • 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 Christmas Disease

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