Haemophilia

Question 1

What is haemophilia. (2)

Answer 1

An inherited disorder of coagulation.

X-linked recessive.

Question 2

Who is affected by haemophilia. (2)

Answer 2

Only males suffer from the disease.

Females are only carriers.

Question 3

What is deficient in haemophilia A.

Answer 3

Deficiency of Factor VIIIc.

Question 4

What is deficient in haemophilia B.

Answer 4

Deficiency of Factor IX.

Question 5

What are the symptoms of haemophilia. (7)

Answer 5

Depends on the severity, and is often early in life or after surgery/trauma.
Haemarthrosis (bleeding into joints).
Haematomas (from bleeding into muscles).
Secondary arthritis.
Spontaneous soft tissue bleeds (ie psoas, gastroncnemius).
GI bleeding.
Haematuria.
Loin pain.

Question 6

What are the blood findings in a patient with haemophilia A. (6)

Answer 6

Increased APTT.
No change in PT or vWF or platelets.
Lack of Factor VIII.
Bleeding time is normal.

Question 7

How is haemophilia inherited.

Answer 7

X linked.

Question 8

What factor is normal in haemophilia A.

Answer 8

vWF.

Question 9

What is the prevalence of haemophilia A.

Answer 9

1 in 10,000 males.

Question 10

What problem is more common in people who suffer from haemophilia than in the general population.

Answer 10

Cerebra haemorrhage.

Question 11

What is a common complication of haemophilia A.

Answer 11

Up to 30% of people with severe haemophilia will, during their lifetime, develop antibodies to factor VIII.

Question 12

What is another name given to haemophilia B.

Answer 12

Christmas disease.

Question 13

What is deficient in haemophilia B.

Answer 13

There is a lack of Factor IX.

Question 14

In patients with haemophilia infected with HIV, what common symptom does not occur.

Answer 14

Kaposi’s sarcoma.

Question 15

What is the incidence of haemophilia B.

Answer 15

1 in 30,000 males.

Question 16

What is a fun fact about haemophilia B.

Answer 16

It was the type of haemophilia affecting the Russia royal family.

Question 17

What is a complication of bleeding into the muscles. (2)

Answer 17

Can lead to nerve palsies and compartment syndrome.

Question 18

What is acquired haemophilia.

Answer 18

A bleeding diathesis causing big mucosal bleeds in males and females.

Question 19

What is acquired haemophilia caused by.

Answer 19

Caused by suddenly appearing auto-antibodes that interfere with factor VIII>

Question 20

What is the half life of factor VIII.

Answer 20

12 hours.

Question 21

What are the characteristics of severe haemophilia.

Answer 21

Spontaneous haemarthroses and muscle haematomas.

Question 22

What are the characteristics of moderate haemophilia.

Answer 22

Mild trauma or surgery causes bleeding.

Question 23

What are the characteristics of mild haemophilia.

Answer 23

Major injury or surgery results in excess bleeding.

Question 24

What joints are usually bled into in haemophilia. (4)

Answer 24

Usually the large joints:
Knees. 
Elbows. 
Ankles. 
Hips.