Always inquire whether patient has known inhibitors - may be refractory to conventional treatment
If so, obtain hematology consult before treatment
If no known inhibitors, and patient not improving after replacement, order mixing study
PTT will not correct if inhibitors present
If so, obtain hematology consult before treatment
If no known inhibitors, and patient not improving after replacement, order mixing study
PTT will not correct if inhibitors present
PTT will not correct if inhibitors present
Suspected bleeding into muscles or joints
Trauma to head or neck
Any new or concerning headache (spontaneous or traumatic)
Trauma with potential for internal bleeding
Prior to any planned invasive procedure or surgery
Gastrointestinal bleeding
Acute fractures, dislocations and sprains
Major bleeding (GI, CNS, large muscle, trauma) requires factor replacement level 80-100%
Moderate bleeding (soft tissue, small muscle, joint) requires 30-50%
Diagnosis unknown
Give FFP (contains VIII and IX)
Each bag raises factor levels by 3-5%
Severe mechanism of injury (head, neck trauma), even without evidence of bleeding<unk>  is factor deficiency, not platelet deficiency/malfunction so initial hemostasis may be achieved but clot stabilization will not persist
Delayed bleeding is a serious risk, so factor replacement must occur immediately
Give FFP (contains VIII and IX)
Each bag raises factor levels by 3-5%<unk>  is factor deficiency, not platelet deficiency/malfunction so initial hemostasis may be achieved but clot stabilization will not persist
Delayed bleeding is a serious risk, so factor replacement must occur immediately
Dose of Factor VIII = weight (kg) x % increased desired x 0.5
After initial correction give half this dose q8-12hr
1 IU/kg will increase the plasma concentration by 2%
For severe bleeding, desired factor level is 80-100%.
For less severe bleeding, the desired factor level is 30-50%
Desmopressin
May be sufficient in patients with mild bleeding
0.3mcg/kg IV over 15-30min
After initial correction give half this dose q8-12hr
1 IU/kg will increase the plasma concentration by 2%
For severe bleeding, desired factor level is 80-100%.
For less severe bleeding, the desired factor level is 30-50%
May be sufficient in patients with mild bleeding
0.3mcg/kg IV over 15-30min
Dose of Factor IX = weight (kg) x % increase desired
After initial correction give half this dose 24 hr later
1 IU/kg will increase the plasma concentration by 1%
For severe bleeding, desired factor level is 80-100%.
For less severe bleeding, the desired factor level is 30-50%
After initial correction give half this dose 24 hr later
1 IU/kg will increase the plasma concentration by 1%
For severe bleeding, desired factor level is 80-100%.
For less severe bleeding, the desired factor level is 30-50%
For isolated oral mucosal bleeding consider antifibrinolytic agents (e.g. - TXA)
Antibody inhibitors to factor therapy more common in Factor concentrates and rare in recombinant factor therapy
Treatment should be in consultation with hematologist
In major bleeding, rVII has been suggested as potential salvage therapy for patients with inhibitors to recombinant factor
Activated Prothrombin Complex Concentrate Dosing: 75 units/kg
FEIBA (Immuno)
Recombinant Activate VII Dosing: 90ug/kg every 2-3 hours
Several studies suggest treatment with higher dosing of Recombinant Activate VII initially
270 mcg/kg dosing
Separate further dosing by at least 6 hours
270 mcg/kg dosing
Separate further dosing by at least 6 hours
Hemophilia
Too few
Von Willebrand disease
Thrombocytopenia
Factor V Leiden
Nonfunctional
Hereditary hemorrhagic telangiectasia
Aspirin