Bleeding Disorders + Thrombophilia Flashcards

1
Q

What is the most common cause of primary haemostatic failure?

A

Thrombocytopenia (usually acquired)

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2
Q

What are the two main mechanisms of thrombocytopenia?

A

Reduced production –> marrow problem

Increased destruction –> DIC, ITP, hypersplenism

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3
Q

What might cause function defects in platelets?

A

Drugs e.g. aspirin, NSAIDs

Renal failure

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4
Q

How is vWF deficiency inherited?

A

Autosomal dominant

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5
Q

Which conditions may cause a multiple factor deficiency?

A

Liver failure
Vitamin K deficiency / warfarin therapy
DIC

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6
Q

How would PT and APTT be affected by a multiple factor deficiency?

A

Both would be prolonged

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7
Q

Where are coagulation factors synthesised?

A

Hepatocytes (reduced in liver failure)

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8
Q

Which factors are carboxylated by vitamin K?

A

2, 7, 9, 10

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9
Q

What are the causes of vitamin K deficiency?

A
Poor dietary intake
Malabsorption
Obstructive jaundice (bile required for absorption)
Warfarin (vitamin K antagonist)
Haemorrhagic disease of newborn
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10
Q

What is DIC?

A

Excessive + inappropriate activation of the haemostatic system –> primary, secondary + fibrinolysis

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11
Q

What are the features of DIC?

A

Microvascular thrombus formation –> end organ failure

Clotting factor consumption –> bruising, purpura + generalised bleeding

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12
Q

What are some causes of DIC?

A

Sepsis
Obstetric emergencies
Malignancies
Hypovolaemic shock

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13
Q

How is DIC managed?

A

Treat underlying cause
Platelet + plasma transfusions
Fibrinogen replacement

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14
Q

What is the pattern of inheritance of haemophilia?

A

X-linked

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15
Q

Which type of haemophilia is most common?

A

Haemophilia A

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16
Q

What causes the bleeding in haemophilia A?

A

Factor 8 deficiency

17
Q

What causes the bleeding in haemophilia B?

A

Factor 9 deficiency

18
Q

What are the features of haemophilia?

A

Haemarthrosis
Haematoma in muscles
Intracranial haemorrhage
Prolonged bleeding after procedure/dental work

19
Q

Which blood result would be suggestive of haemophilia?

A

Isolated prolonged APTT

PT not affected

20
Q

How is haemophilia A managed?

A
Avoid contact sport
IV factor 8 concentrate
- weekly for prophylaxis or until bleeding stops if acute
Desmopressin 
- for acute bleeds in mild haemophilia
21
Q

What is INR?

A

Standardised norm for PT

22
Q

How do the clots differ between arterial and venous thrombosis?

A

Arterial:
- high pressure, atherosclerosis
- platelet rich thrombus
Venous:
- low pressure so platelets not activated
- coagulation cascade –> fibrin rich clot

23
Q

Which drugs are used to treat arterial thrombosis?

A

Aspirin + other anti-platelets

24
Q

Which drugs are used to treat venous thrombosis?

A

Heparin, warfarin + DOACs

25
Q

What is thrombophilia?

A

Disorder (inherited or acquired) which predisposes to thrombosis

26
Q

Give some examples of hereditary thrombophilias

A
Factor 5/Leiden 
Prothrombin 20210 mutation
Antithrombin deficiency
Protein C deficiency
Protein S deficiency
27
Q

When should you consider screening for inherited thrombophilias?

A

Venous thrombosis < 45 years old
Recurrent venous thrombosis
Usual venous thrombosis
FHx of venous thrombosis/thrombophilia

28
Q

Give an example of an acquired thrombophilia?

A

Antiphospholipid antibody syndrome

29
Q

What are the features of antiphospholipid antibody syndrome?

A

Recurrent arterial and venous thrombosis
Recurrent foetal loss
Mild thrombocytopenia

30
Q

Which drugs are used to manage antiphospholipid antibody syndrome?

A

Aspirin + warfarin

–> as activation of both primary + secondary haemostasis