x linked recessive causing defective VIII:c,
Replace c cryoprecipitate of factor VIII 1 unit=100 units VIII
1 cc of FFP=1 unit VIII and less of IX
dDAVP raises factor VIII by causing release from endothelial cells, It has ADH-like effects. Dose: 0.3 mcg/kg.
Number of units=0.5 x (Desired Factor Level) x (Weight in Kg) (1/2 life of 12 hours) (Each unit of factor/kg raises levels by 2%)
Raise to 100% in head injury, mult. Trauma, neck/tongue/throat hematoma, ABD pn/GI bleed, eye trauma.
VIII-plasma or recombinant
evels for Hem A/B
- Joint/Tissue-50% A, 30% B impending joint will present with bubbly feeling, tingling, warmth
- Mouth-correct 50/30%, Amicor 100 mg/kg (max 24 g/day) Q6 x 1 week
- Epistaxis-local measures the factor 50/30
- Iliopsoas hemorrhage-100/80% then maintain above 40
- Urinary-vigorous hydration, if still bleeding, replace factor. Prednisone 2mg/kg divided TID x 2 days
- Suturing-replace factor first
- Trauma-100% replacement
- Fractures-100/80 prior to splinting
- Abd Pain-100/80
10 minute post transfusion, get PTT and factor level
Hemophilia B (Christmas)
Factor IX, replace c factor
1 x Factor Level x Kg (1/2 life of 24 hours)
(Benefix is only 80% factor IX so multiply by 1.25 instead of 1)
IX-older plasma derived can be thrombogenic, use recombinant
von Willebrand’s Disease
Autosomal (usually dominant)
Presents with mucocutaneous bleeding, commonly epistaxis, gi bleeds, and prolonged menorrhagia.
Patients will have prolonged PTT, increased bleeding time, Normal PT and Platelet count.
1 bag cryo per 10 kg or FFP in emergency
Consider if prolonged vaginal bleeding in menarche or in women in the perimenopausal period. Estrogen and progesterone raise vWF level. When Anovulatory cycles are present, may see prolonged bleeding.
Treat with dDAVP, Factor VIII concentrates with high vWF levels (Humate-P or Koate-HS), Cryoprecipitate (10 units/kg), FFP