Haemophilia Flashcards
What is haemophilia. (2)
An inherited disorder of coagulation.
X-linked recessive.
Who is affected by haemophilia. (2)
Only males suffer from the disease.
Females are only carriers.
What is deficient in haemophilia A.
Deficiency of Factor VIIIc.
What is deficient in haemophilia B.
Deficiency of Factor IX.
What are the symptoms of haemophilia. (7)
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.
What are the blood findings in a patient with haemophilia A. (6)
Increased APTT.
No change in PT or vWF or platelets.
Lack of Factor VIII.
Bleeding time is normal.
How is haemophilia inherited.
X linked.
What factor is normal in haemophilia A.
vWF.
What is the prevalence of haemophilia A.
1 in 10,000 males.
What problem is more common in people who suffer from haemophilia than in the general population.
Cerebra haemorrhage.
What is a common complication of haemophilia A.
Up to 30% of people with severe haemophilia will, during their lifetime, develop antibodies to factor VIII.
What is another name given to haemophilia B.
Christmas disease.
What is deficient in haemophilia B.
There is a lack of Factor IX.
In patients with haemophilia infected with HIV, what common symptom does not occur.
Kaposi’s sarcoma.
What is the incidence of haemophilia B.
1 in 30,000 males.
What is a fun fact about haemophilia B.
It was the type of haemophilia affecting the Russia royal family.
What is a complication of bleeding into the muscles. (2)
Can lead to nerve palsies and compartment syndrome.
What is acquired haemophilia.
A bleeding diathesis causing big mucosal bleeds in males and females.
What is acquired haemophilia caused by.
Caused by suddenly appearing auto-antibodes that interfere with factor VIII>
What is the half life of factor VIII.
12 hours.
What are the characteristics of severe haemophilia.
Spontaneous haemarthroses and muscle haematomas.
What are the characteristics of moderate haemophilia.
Mild trauma or surgery causes bleeding.
What are the characteristics of mild haemophilia.
Major injury or surgery results in excess bleeding.
What joints are usually bled into in haemophilia. (4)
Usually the large joints: Knees. Elbows. Ankles. Hips.
