Hemophilia A: Factor VIII Deficiency Quiz

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10 Questions

What is the main factor deficiency seen in hemophilia?

Factor VIII

Which coagulation factor is responsible for initiating thrombin formation in hemophilia?

Factor VIII

What kind of mutations can be found on the Factor VIII locus in hemophilia?

Severe molecular defects (large deletions, intron 22 inversions, nonsense mutations) or milder molecular defects like point mutations

What is the consequence of lacking Factor VIII as a cofactor in hemophilia?

Slows the rate of FXa generation

Why does bleeding occur in hemophilia?

Defective fibrin stabilization due to inadequate fibrin generation

Where does bleeding commonly occur in patients with hemophilia?

Load-bearing joints (ankles, knees, elbows) and intracapsular bleeds

What is the commonest cause of death in hemophilia?

CNS bleeding

What does a full haemogram typically show in hemophilia?

Normal to low Hb/hct values, normal platelet count

What symptoms may patients with hemophilia present with depending on their Factor VIII activity level?

Easy bruising, inadequate clotting of traumatic or mild injury, spontaneous hemorrhage in severe cases

What is the role of Factor VIII in the coagulation cascade?

Initiate thrombin formation for adequate fibrin generation

Test your knowledge on Hemophilia A, an X-linked inherited bleeding disorder affecting 1 in 5000 males. Learn about the deficiency or complete lack of Factor VIII, a crucial pro-coagulant cofactor in the coagulation cascade.

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