112 Coagulation Flashcards
defined as strategy for maintaining the missing clotting factor level at about 1% of higher on a regular basis in order to prevent bleeds, esp the onset of hemarthrosis
primary prophylaxis
how is primary prophylaxis done
regular infusion of FVIII (3 days/ week ) or FIX (2 days/ week)
One unit of FVIII per KBW increases the plasma FVIII level by how much
2%
True or false. FIX replacement are different from those for FVIII
true. because FIX recovery after infusion is usually only 50% as per predicted value
what is the half life of FVIII
8-12 hours thus requires injections twice a day to maintain therapeutic levels
what is the half of FIX
about 24 hours thus a once-a-day injection is sufficient
enriched with FVIII protein
cryoprecipitate
how much FVIII does each unit of cryoprecipitate contain
about 80 IU of FVIII
synthetic vasopressin analog that causes transient rise in FVIII and vWF but NOT FIX
DDAVP
how is DDAVP given
0.3 ug/KBW, over a period of 20 min is expected to raise FVIII by two to threefold over baseline, peaking between 30- 60 min after infusion
how is antifibrinolytic drugs given to bleeding gums and GI in hemophiliacs
tranexamic acid 25 mg/kg 3-4x a day maximum loading load of 200 mg/kg or 1 gram followed by 100 mg/kg maximum 30 g/d every 6 hours
true or false. tranexamic acid is not given to control hematuria as risk for occlusive clot in the lumen of the GUT
True.
major complication of hemophilia treatment
formation of alloantibodies to FVIII or FIX
laboratory tests required to confirm the presence of an inhibitor
APTT with a mix
interpretation of mixing studies. normal? prolonged?
normal: hemophilia
prolonged: inhibitors
most effective therapy in hemophiliacs with inhibitors
immune tolerance induction (ITI)
most prevalent primary liver cancer in hemophiliac
hepatocellular cancer
in hemophiliac carriers when is the risk for bleeding highest?
after delivery as clotting factors rapidly decrease; during pregnancy there is a threefold increase in FVIII
How is clotting factor infusion done in to prevent imminent bleeding among patients carriers of hemophilia
infusion of factor concentrate to 50-70% level for 3 days in the setting of vaginal delivery and up to 5 days for cesarean delivery
what is the half life of FXI
40- 70 hours
how is FXI deficiency addressed
infusion of FFP at 15-20 ml/ k to maintain trough levels ranging from 10 to 20%
clinicopathologic syndrome characterized by widespread intravascular thrombin fibrin formation
DIC
common cause of DIC in pregnant women
abruptio placentae or amniotic fluid embolism
severe form of DIC resulting from thrombosis of extensive areas of the skin
purpura fulminans
