Bleeding Disorders

Question 1

Which blood components are most likely to be responsible for bleeding disorders?

Answer 1

1 - Platelets

2 - Von Willebrand Factor

3 - Coagulation factors

Question 2

What are important points that should be established in a bleeding disorder history taking?

Answer 2

1 - Do they actually have a bleeding disorder

2 - How severe is the disorder

3 - What is the pattern of bleeding

4 - Is the condition congenital or acquired

5 - What is the mode of inheritance

Question 3

What are common features that are present when taking a history from a patient with a bleeding disorder?

Answer 3

1 - Easily bruised

2 - Epistaxis

3 - Post-surgical bleeding

4 - Menorrhagia

5 - Post-partum haemorrhage

6 - Post-trauma bleeding

Question 4

When considering the severity of a bleeding disorder, what needs to be considered?

Answer 4

How appropriate is the bleeding? I.e. bleeing from being stabbed in the chest (very appropriate) compared to bleeding standing up straight (very inappropriate)

Question 5

What is the pattern of bleeding associated with platelets and VWF?

Answer 5

They bleed onto surfaces (mucosa, menorrhagia, GI)

image - non-blanching petechiae

Question 6

What is the bleeding pattern associated with coagulation factors?

Answer 6

They bleed into spaces (hinge joints, muscles, CNS)

Question 7

What type of inheritance does Haemophilia A and B have?

Answer 7

X-linked recessive

Question 8

What does a X-linked recessive transmission indicate about the likely disease status of the parents?

Answer 8

A father with the defective gene for haemophilia will always be affected (display the phenotype)

Only sometimes will a female be affected if they have 1 copy of the mutation, however having 2 copies of the mutation (homozygous) will definitely result in the phenotype being displayed

Question 9

How do the phenotypes of Haemophilia A and B compare?

Answer 9

They are identical

Question 10

What is the occurence rate for Haemophilia A and B?

Answer 10

Haemophilia A - 1 in 10,000

Haemophilia B - 1 in 60,000

Question 11

The residual level of which clotting hormones controls the severity of the haemophilia?

Answer 11

Coagulation Factor

Question 12

A clotting factor concentration of <1% will result in what severity of haemophilia?

Answer 12

Severe

Question 13

A clotting factor concentration of 1-5% will result in what severity of haemophilia?

Answer 13

Moderate

Question 14

A clotting factor concentration of 5-30% will result in what severity of haemophilia?

Answer 14

Mild

Question 15

What are the clinical features of haemophilia?

Answer 15

Haemarthrosis - Bleeding into joints (usually hing joints)

Muscle Haematoma

CNS bleeding

Retroperitoneal bleeding

Post surgical bleeding

Question 16

What are the clinical complications of haemophilia?

Answer 16

1 - Synovitis

2 - Chronic haemophiliac arthropathy

3 - Neurovascular compression

4 - Stroke

Question 17

How is haemophilia diagnosed?

Answer 17

1 - Clinical picture

2 - Prolonged APTT

3 - Normal PT

4 - Reduced Factor VIII or IX

Question 18

If someone with haemophilia starts to bleed, how should they be treated?

Answer 18

Give them Factor VIII (A) or IX (B)

Question 19

Apart from replacing the deficient coagulation factors, what are some other treatment options available for haemophilia bleeds?

Answer 19

Desmopressin

Tranexamic acid

Question 20

What are the general treatment options for haemophilia?

Answer 20

Splints (to help joint pain)

Physio

Analgesia

Joint replacement

Question 21

What are the common complications from treatment of haemophilia?

Answer 21

1) Viral infection from receiving blood - HIV, HBV, HCV
2) Antibodies to Factor VIII
3) DDAVP - MI’s

Question 22

What is the occurence rate for von Willebrand Disease?

Answer 22

1 in 200

Question 23

What type of bleeding occurs in vWD?

Answer 23

Platelet type bleeding (onto surfaces)

Question 24

What causes vWD?

Answer 24

Abnormalities in vWF

Question 25

How is vWD inherited?

Answer 25

Autosomal (from chromosomes)

Question 26

What are the different types of vWF deficiency seen in vWD?

Answer 26

Type 1 - quantitative deficiency

Type 2 - qualitative deficiency

Type 3 - severe deficiency

Question 27

How is vWD treated?

Answer 27

vWF concentrate

Desmopressin

Tranexamic acid

Question 28

What are common acquired bleeding disorders or causes of bleeding disorders?

Answer 28

1 - Thromocytopenia

2 - Secondary to liver failure

3 - Secondary to renal failure

4 - Disseminated Intravascular Coagulation

5 - Anti-coagulant drugs (warfarin, Heparin, clopidogrel etc.)

Question 29

What is thrombocytopenia and how is it diagnosed?

Answer 29

Decreased production of thrombocytes(platelets)

Diagnosis - Low platelet count

Question 30

What are the clinical features of thrombocytopenia?

Answer 30

1 - Petechia

2 - Ecchymosis

3 - Mucosal bleeding

Question 31

What is the cause of bleeding disorders frm liver disease?

Answer 31

The balance between coagulants and anti-coagulants produced by the liver is distrubed in liver disease

Question 32

In patients with bleeding disorders from liver disease where are they most likely to loose blood from?

Answer 32

Varices

Question 33

What are the key deficiencies noted in bleeding disorders caused from liver disease?

Answer 33

Vitamin K

Fibrinogen

Question 34

How are bleeding disorders caused by liver disease treated?

Answer 34

Vitamin K - IM injection

Replacement FFP

Question 35

What is haemorrhagic disease of the newborn and how is it treated?

Answer 35

Disturbance in ability to effectively coagulate caused by a vitamin K deficiency

Treatment - Vit K injection at birth