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Haematology > Bleeding Disorders > Flashcards

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
Q

What is haemophilia?

A

An x-linked hereditary disorder in which abnormally prolonged bleeding recurs episodically at one or a few sites on each occasion

26
Q

Which haemophilia is more common between A and B?

A

A (factor 8 deficiency)

27
Q

What is the main consequence of haemophilia?

A

Formation of “target joints”

Iron in blood will irritate the synovium forming neovascularization which are fragile and prone to bursting

28
Q

What aspect of secondary haemostasis will haemophilia affect?

A

Loss of amplification

29
Q

Which screening test will be affected in haemophilia?

A

ONLY APTT

30
Q

What are the clinical features of severe haemophilia?

A

Recurrent hemarthrosis
Recurrent soft tissue bleeds
Prolonged bleeding post dental extraction

31
Q

How is severe haemophilia treated?

A

IV injections of clotting factor 8/9 every 2 days

Haematology (31 decks)

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