Hemophilia B, or factor
IX deficiency(factor
9 deficiency).
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
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 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:
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)
Human immunodeficiency virus (HIV), Hepatitis B, or Hepatitis C infection by transfusion
