3.09 SGD Hemophilia

Question 1

Hemophilia A is also known as __ or __.

Answer 1

Factor VIII deficiency or Classic hemophilia

Question 2

Inheritance pattern of hemophilia

Answer 2

X-linked recessive

Question 3

T/F. It is dangerous for a person with hemophilia to get wounds because they release an increased volume of blood per unit time as compared to non-hemophiliacs.

Answer 3

False. People with hemophilia bleed longer, but don’t necessarily bleed more than people without hemophilia.

Question 4

T/F. There is the absence of the formation of the platelet plug in hemophilia.

Answer 4

False. Formation of the platelet plug happens, but it is deficient. Fibrin clot that follows is also delayed.

Question 5

Sign/symptom of hemophilia that pertains to bleeding into joint spaces; it usually involves large joints, knees, elbows

Answer 5

Hemarthrosis

Question 6

Sign/symptom of hemophilia that pertains to bleeding into soft tissues and muscles

Answer 6

Hematoma

Question 7

Sign/symptom of hemophilia that pertains to blood in the urine

Answer 7

Hematuria

Question 8

Typical complication that arises when hemarthrosis is not addressed

Answer 8

Joint stiffness/damage

Question 9

Which part of the coagulation cascade is affected by having a deficiency of FVIII? What does this cause?

Answer 9

Activation of FX in the intrinsic pathway. It results in the formation of fibrin deficient clots which makes coagulation prolonged and the clot more unstable

Question 10

Expected result of PTT in hemophilia

Answer 10

Prolonged, greater than normal/standard range

Question 11

Which factor/s in the results for factor assay will have abnormal results?

Answer 11

Factor VIII

Question 12

Possible X-ray result

Answer 12

Pleural effusion present –> blood extravasation

Question 13

What are the results of the differential tests vWF, FVIII and bleeding time?

Answer 13

vWF: normal
FVIII: low
bleeding time: normal

Question 14

Treatment that involves supplementing FVIII

Answer 14

Factor Replacement Therapy

Question 15

Treatment that stimulates release of stored coagulation factor VIII and von Willibrand factor in the body

Answer 15

Hormone desmopressin

Question 16

1. Treatment that involves the use of drugs that help to hold a clot in place once it has formed.
2. What mechanism does it use/what does it do?

Answer 16

1. Antifibrinolytics (Tranexamic acid and Aminocaproic acid)

2. Inhibits activity of plasmin, an enzyme that dissolves blood clots

Question 17

Treatment that involves transfusion of regular Factor VIII every 2-3 days

Answer 17

Prophylactic Therapy

Question 18

Why is Heparin considered a contraindicator?

Answer 18

Because it accelerates inhibition of antithrombin to thrombin, thereby inhibiting clotting/coagulation

Question 19

Why is Warfarin considered a contraindicator?

Answer 19

Because it is a vitamin K antagonist, which plays an important role in the posttranslational synthesis of factors II, VII, IX, X

Question 20

What other examples of drugs aside from Heparin and Warfarin are considered contraindicators?

Answer 20

Aspirin (anti-thrombotic)
Ibuprofen
Naproxen Sodium
Some herbal medicines