Indications for Factor Replacement Therapy
1. Suspected bleeding into a joint or muscle.
2. Any significant injury to the head, neck, mouth or eyes or evidence of bleeding in these
areas.
3. Any new or unusual headache, particularly one following trauma.
4. Severe pain or swelling at any site.
5. All open wounds requiring surgical closure, wound adhesive, or steri-strips.
6. History of an accident or trauma that might result in internal bleeding.
7. Any invasive procedure or surgery.
8. Heavy or persistent bleeding from any site.
9. Gastrointestinal bleeding leading to moderate to severe anemia.
10. Acute fractures, dislocations and sprains.
11. Heavy menstrual bleeding leading to moderate to severe anemia or volume instability.
Treatment
Hemophilia A without Inhibitor
➤ The treatment of choice for individuals with hemophilia A (factor VIII
deficiency) is recombinant factor VIII or the patient's product of choice.
Plasma-derived concentrate is a suitable alternative in an emergency
situation when recombinant factor VIII is not available. Cryoprecipitate
and fresh frozen plasma are no longer recommended for treatment of
individuals with hemophilia A.
➤ When bleeding is severe, the appropriate dose of factor VIII is 50 units/kg 50 units/kg.
This should result in a factor VIII level of 80-100%.
➤ In individuals with hemophilia A receiving prophylaxis with emicizumab
who present with acute bleeds, factor VIII should be given, as above. While
emicizumab is effective in preventing bleeds (prophylaxis), it is ineffective
in treating acute bleeding events. No adjustment in factor VIII concentrate
dosing is recommended for those patients on emicizumab.
Mild Hemophilia A with Non-Life or Limb Threatening Bleeding
➤ Individuals with mild hemophilia A (baseline factor VIII greater than 5% and
less than 50%) who are experiencing non-life or limb-threatening bleeding
may respond to desmopressin (DDAVP, see Drug table). This therapy should
be used only if there is documentation in the medical record demonstrating
a hemostatic response to this medication. Otherwise, treatment is the
same as for other individuals with hemophilia A.
