Pharmacology Of Disorders Of Hemostasis And Coagulation

Question 1

Two pathways of coagulation cascade

Answer 1

Intrinsic pathway: slower process that begins in circulation with factor 12

Extrinsic pathway: fast process that begins with release of tissue factor

Question 2

Result of all coagulation cascade pathways

Answer 2

Prothrombin -> thrombin

Question 3

Vitamin K-dependent coagulation factors

Answer 3

2, 7, 9, 10

Question 4

Thrombin-sensitive coagulation factors

Answer 4

5,8,13 and fibrinogen

Question 5

Contract activation coagulation factors

Answer 5

11,12, prekallikrein and kininogen

Question 6

Hemophilia

Answer 6

A bleeding disorder that results from congenital deficiency in plasma coagulation

3 types

Question 7

Hemophilia A

Answer 7

1/5000 males only

Deficiency in factor 8

Question 8

Hemophilia B

Answer 8

Affects 1/30000 males or females

Deficiency in factor 9

Question 9

Hemophilia C

Answer 9

Affects 1/100000 in both males and females

Deficiency in factor 11

Question 10

Clinical manifestations of hemophilia

Answer 10

Palatable bruising

Hemarthrosis and joint pain

Muscle hemorrhages

Excessive bleeding after surgery or trauma
* bleeding depends on severity*

Question 11

Factor plasma levels and the ranges for severity of hemophilia

Answer 11

Normal = 0.5-1.5 mL

Mild = 0.05-0.4 mL

Moderate = 0.01- 0.05 mL

Severe = <0.01 mL

Question 12

Recombinant factor vs plasma derived factors

Answer 12

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

Question 13

Why are hep B and A vaccines highly recommended for hemophilia patients

Answer 13

1 possible infection via recombinant of plasma derived treatments is Hep B/A

Question 14

Low-titer inhibitors vs high-titer inhibitors

Answer 14

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

Question 15

Inhibitors of hemophilia in patients

Answer 15

Patient develops antibodies to the administered factors. Can either skip by the factors and administer prothrombin or give the dose more often

Question 16

Von willebrand disease

Answer 16

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

Question 17

Clinical manifestation of vWF disease

Answer 17

Mucocutaneous bleeding

Common frequent Nose bleeds and bleeding of gums

Epistaxis

Easy brushing

Postoperative bleeding

NO blood in muscle joints

Question 18

Immune thrombocytopenic purpura (ITP)

Answer 18

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*