Bleeding Disorders Flashcards

1
Q

What are the anticoagulant defences in the normal haemostatic system?

A

Protein C and S

Anti-thrombin

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2
Q

What can cause failure of platelet plug formation?

A

Vascular - collagen issues
Platelets - thrombocytopenia, reduced function
Von Wilbrand Factor

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3
Q

What can cause vascular abnormalities that result in failure of primary haemostasisi?

A

Marfan’s syndrome (ghent criteria)
Vasculitis
HSP
Vit C deficiency - scurvy

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4
Q

What can cause thrombocytopenia?

A

Marrow problem; pancytopenia

Increased destruction - ITP

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5
Q

What is the commonest cause of thrombocytopenia?

A

Immune thrombocytopenic purpura

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6
Q

What can cause peripheral platelet destruction?

A

Coagulopathy; DIC
Autoimmune; ITP
Hypersplenism; liver dx, lymphoma

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7
Q

What blood results will DIC show?

A

Issue with secondary and primary haemostasis
Prolonged prothrombin and APTT
Thrombocytopenia
Raised d-dimer

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8
Q

What can cause an acquired platelet functional defect?

A

Drugs; aspirin, NSAIDs

Renal failure

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9
Q

What is vWF deficiency?

A

AD disease
Common
Variable severity
Failure of platelet sticking

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10
Q

How will a primary haemostatic problem present?

A

Bleeding from mucosal surfaces; nose bleeds, bleeding gums, menorrhagia, increased bleeding post operations

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11
Q

What can cause failure in secondary haemostasis (fibrin clot formation)?

A
Multiple clotting factor deficiencies:
Acquired
DIC
Single clotting factor: 
Haemophilia
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12
Q

What is the difference between haemophilia A and haemophilia B?

A
A = factor 8 
B = factor 9
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13
Q

What can cause multiple factor deficiencies?

A

Liver failure
Vit K deficiency/ warfarin therapy
DIC

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14
Q

What will multiple factor deficiencies lead to in terms of screening tests?

A

Prolonged PT time

Prolonged APTT

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15
Q

Where are all clotting factors synthesized?

A

Hepatocytes

Of all; factor 8 is least affected by liver failure

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16
Q

What clotting factors are carboxylated by vitamin K?

A

2,7,9,10

1972

17
Q

What is the source of vitamin K?

A

Diet
Intestinal synthesis
Absorbed in upper intestine
Requires bile salts for absorption - fat soluable vitamin

18
Q

What can cause vitamin K deficiency?

A
Poor dietary intake - green leafy veg
Malabsorption 
Obstructive jaundice
Vit K antagonists (warfarin) 
Haemorrhagic disease of newborn
19
Q

In what part of the small bowel specifically is vitamin K absorbed?

A

Jejunum and ileum

20
Q

How can haemorrhagic disease of the newborn be prevented?

A

All newborn babies are given a once off injection of vit K at birth

21
Q

What is DIC?

A

Excessive and inappropriate activation of the haemostatic system - primary, secondary and fibrinolysis
Microvascular thrombus formation; end organ failure
Clotting factor consumption - bruising, purpura and generalised bleeding

22
Q

What clotting factor has the shorted half life and therefore the screening test is most sensitive for warfarin therapy and liver failure?

A

7; prothrombin time

23
Q

What can cause DIC?

A

Sepsis; tissue damage from bacterial endo/exo toxin
Obstetric emergencies - placenta rich in phospholipid and tissue factor
Malignancy - adenocarcinoma of bowel and prostate
Hypovolemic shock

24
Q

How is DIC treated?

A

Treat underlying cause
Platelet transfusion
FFP
Cryoprecipitate

25
What is haemophilia?
An x-linked hereditary disorder in which abnormally prolonged bleeding recurs episodically at one or a few sites on each occasion
26
Which haemophilia is more common between A and B?
A (factor 8 deficiency)
27
What is the main consequence of haemophilia?
Formation of "target joints" | Iron in blood will irritate the synovium forming neovascularization which are fragile and prone to bursting
28
What aspect of secondary haemostasis will haemophilia affect?
Loss of amplification
29
Which screening test will be affected in haemophilia?
ONLY APTT
30
What are the clinical features of severe haemophilia?
Recurrent hemarthrosis Recurrent soft tissue bleeds Prolonged bleeding post dental extraction
31
How is severe haemophilia treated?
IV injections of clotting factor 8/9 every 2 days