Pharmacology Of Disorders Of Hemostasis And Coagulation Flashcards
Two pathways of coagulation cascade
Intrinsic pathway: slower process that begins in circulation with factor 12
Extrinsic pathway: fast process that begins with release of tissue factor
Result of all coagulation cascade pathways
Prothrombin -> thrombin
Vitamin K-dependent coagulation factors
2, 7, 9, 10
Thrombin-sensitive coagulation factors
5,8,13 and fibrinogen
Contract activation coagulation factors
11,12, prekallikrein and kininogen
Hemophilia
A bleeding disorder that results from congenital deficiency in plasma coagulation
3 types
Hemophilia A
1/5000 males only
Deficiency in factor 8
Hemophilia B
Affects 1/30000 males or females
Deficiency in factor 9
Hemophilia C
Affects 1/100000 in both males and females
Deficiency in factor 11
Clinical manifestations of hemophilia
Palatable bruising
Hemarthrosis and joint pain
Muscle hemorrhages
Excessive bleeding after surgery or trauma
* bleeding depends on severity*
Factor plasma levels and the ranges for severity of hemophilia
Normal = 0.5-1.5 mL
Mild = 0.05-0.4 mL
Moderate = 0.01- 0.05 mL
Severe = <0.01 mL
Recombinant factor vs plasma derived factors
Recombinant: works slightly better and has a very low infection rate
- expensive
Plasma-derived: works well but slightly worse than recombinant and has a chance or infection
- cheaper
Why are hep B and A vaccines highly recommended for hemophilia patients
1 possible infection via recombinant of plasma derived treatments is Hep B/A
Low-titer inhibitors vs high-titer inhibitors
Low: treated by giving 2-3x the normal doses and more frequently
High: cant use the factors themself and instead skip cascade and increases concentrations of prothrombin
Inhibitors of hemophilia in patients
Patient develops antibodies to the administered factors. Can either skip by the factors and administer prothrombin or give the dose more often
Von willebrand disease
Deficiency in amount of vWF
- can have 3 types
Function is to be released from endothelium after vessel injuries
- promotes platelet adhesion to collagen fibers and binds to factor 8
- the binding of factor 8 prevents its degradation and brings it to where it needs to go
Clinical manifestation of vWF disease
Mucocutaneous bleeding
Common frequent Nose bleeds and bleeding of gums
Epistaxis
Easy brushing
Postoperative bleeding
NO blood in muscle joints
Immune thrombocytopenic purpura (ITP)
Acquired autoimmune disorder where antibodies are naturally made to destroy your own platelets
Children: follows with severe infections
Adults: follows with chronic disease
- mucocutaneous bleeding and severe low levels of platelets*
