COAGULATION DEFECTS

Question 1

characterized by multiple clotting abnormalities

Vitamin K Deficiency – depresses synthesis of II, VII, IX, X, protein C, protein S

Liver disease/DIC – causes coagulation problems

Answer 1

Acquired Coagulation Deficiencies

Question 2

Caused by problems in the Factor VIII-vWF complex

Deficiency in Factor VIII or Factor VIII procoagulant protein

Answer 2

Hereditary Coagulation Deficiencies

Hemophilia A, Factor VIII-vWF complex

Question 3

required for activation of factor X

Answer 3

Factor VIII

Question 4

multimeric protein that binds to collagen, heparin, platelet membrane glycoproteins Ib-Ix and IIb-IIIa

Answer 4

vW Factor

Question 5

common inherited bleeding disorder (1% of people)

Clinical Signs**
• spontaneous bleeding from mucous membranes
• excessive wound bleeding
• menorrhagia (heavy periods)
• prolonged bleeding time

Answer 5

Von Willebrand’s Disease

Question 6

name this type of Von Willebrand’s Disease –

autosomal dominant - 70% of all cases

o Causes a mild disease
o Reduced penetrance and variable expressivity

Answer 6

Type 1 vWD

Question 7

name this type of Von Willebrand’s Disease –

autosomal dominant, missense mutation

Answer 7

Type 2 vWD

Question 8

name this type of Von Willebrand’s Disease –

– autosomal recessive

o A much more severe disease causing very low levels of vWF
o Testing may resemble hemophilia due to instability of factor VIII

Answer 8

Type 3 vWD

Question 9

caused by low levels of Factor VIII

Cause – X-linked recessive

Answer 9

Hemophilia A

Question 10

factor IX deficiency (“Christmas Disease”)

Cause – X-linked recessive

Answer 10

Hemophilia B