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