Bleeding Disorders

Question 1

What is an epistaxis?

Answer 1

A nosebleed

Question 2

What are some important questions to ask when you suspect a patient may have a bleeding disorder?

Answer 2

• Menorrhagia / post-partum bleeding?
• Post surgical bleeding?
• What sort of trauma provokes bleeding?

Question 3

What are the different patterns of bleeding?

Answer 3

Platelet type abnormalities - mucosal

Coagulation factor deficiencies

Question 4

What sort of bleeding may be seen in platelet type mucosal bleeding?

Answer 4

Epistaxis

Purpura (blood spots on the skin)

Menorrhagia

GI

Question 5

What sites are most affected by coagulation factor deficiency bleeds?

Answer 5

• Articulations / joints (particularly hinge - knee / elbow / ankle)
• Muscle haematoma
• CNS

Question 6

What are petechiae?

Answer 6

Blood spots on the skin, can appear purple / red / brown

Caused by the bursting of blood vessels

Press on them to see if they blanch or not, petechiae won’t blanch as there is no blood vessel for blood to escape into

Question 7

What happens in haemophilic arthropathy?

Answer 7

• Bleeding into the joint spaces
• Iron and haeme taken up by macrophages in the joint causes an inflammatory response
• Inflammation of the synovium prevents the normal repair of the cartilage and the joint, often resulting in end stage joint disease

Question 8

What used to be a very common cause of death in severe haemophilia before coagulant therapies became more advanced?

Answer 8

Intracranial haemorrhage in haemophilia

Question 9

What questions are important when determining if a bleeding disorder is congenital?

Answer 9

Personal history of bleeds following surgeries etc.

Ask about family members who have been affected and the sex of these family members (is the disorder x-linked? can give a clue to the disease)

Question 10

Which haemophilia (A or B) is more common?

Answer 10

A is more common, both quite rare though

Question 11

What determines the severity of haemophilia A and B?

Answer 11

The residual coagulation factor activity

Question 12

How does the percentage of residual coagulation factor activity correlate with severity of haemophilia A & B?

Answer 12

< 1% activity - severe. Unprovoked bleeding occurs

1 - 5% activity - Moderate. Bleed excessively under trauma or after surgery

5 - 30% - Mild. Won’t bleed in most situations but will bleed after surgery or biopsy

Question 13

What are the clinical features of haemophilia A and B?

Answer 13

• Haemoarthrosis
• Muscle Haematoma
• CNS bleeding
• Retroperitoneal bleeding
• Post Surgical Bleeding

Question 14

What are some of the complications that can be involved with haemophilia?

Answer 14

Synovitis

Clinical Haemophilic Arthropathy (often leads to early joint replacement)

Neurovascular Compression (compartment syndromes)

Other conditions of bleeding such as stroke

Question 15

What are some lab tests done for the diagnosis of haemophilia?

Answer 15

APTT - Activated Partial Thromboplastin Time (should be prolonged)

PT - prothrombin time (prolonged)

If APTT prolonged do an assay for Factor VIII & IX

Question 16

How are haemophilia A and B treated?

Answer 16

Coagulation factor replacement