Test 2 Coagulation Factors Flashcards
coagulopathy
any defect in blood clotting -> there is hyper and hypo; hyper leads to pulmonary embolism
Hemophilia
- Excessive bleeding tendency
- X-chromosome linked recessive disorder
- 3 categories: Type A, B, C
Type A Hemophilia
- Classical hemophilia
- 8 out of 10 males who have hemophelia has this
- Defective production or absence of factor VIII
Type B Hemophilia
- occasionally, Christmas disease
- Defective production or absence of factor IX
Type C Hemophilia
- Less common and is a mild bleeding disorder
- Deficiency of factor XI
Where does the extrinsic and intrinsic pathway meet?
- Factor X
- FX activates fibrinogen to firbin
Locations of extrinsic and intrinsic pathway
- Extrinsic: in blood
- Intrinsic: mostly in tissue
Factor VIII
- Antihemophilic factor (AHF)
- glycoprotein
- cofactor in the activation of factor X by factor IXa
- requires Ca+2 and phospholipids
- cDNA encoding for factor VIII is expressed in mammalian cell lines
brand names for rFactor VIII products
- Recombinate
- Xyntha
- Refacto
- Helixate
- Bioclate
- Advat
- Kogenate
properties of rFactor VIII products
- Dosage must be individualized
- Risk of developing antibodies
Recombinant factor IX
- Factor IX is activated by factor VIIa in the extrinsic pathway and by factor XIa in the intrinsic pathway
- Indicated for control and prevention of hemorrhagic events in hemophilia type B patients
properties of rFactor IX products
BeneFix
properties of rFactor IX products
- Activity is about 200 IU/mg
- Contraindicated in patients with known hypersensitivity to mouse, hamster or bovine proteins
- bolus is administered over a 10-15 minute period
Recombinant factor VIIa
- serine protease glycoprotein
- no matter how hard you try to purify factor VII, it gets purified into its active form
- Used when antibodies (inhibitors) develop for factors VIII or IX
production of recombinant factor VIIa
Produced in BHK cells with cDNA containing human factor VIIa gene
brand names for rFactor VIIa products
NovoSeven
properties of rFactor VIIa products
Contraindicated in patients with known hypersensitivity to mouse, hamster or bovine proteins