Bleeding Disorders Flashcards

1
Q

What can cause failure of platelet plug formation? (primary haemostasis)

A

vascular disease
von willebrand disease
platelets - decreased number or reduced function

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2
Q

What can cause decreased function of platelets?

A

acquired:
drugs e.g. aspirin, NSAIDs
renal failure
hereditary: v rare

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3
Q

Why does renal failure cause reduced platelet function?

A

build up of toxins interfere with platelet function

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4
Q

What is thrombocytopenia?

A

decreased number of platelets

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5
Q

What can cause thrombocytopenia?

A

hereditary
acquired:
increased destruction - hypersplenism e.g. liver disease, coagulopathy e.g. DIC, autoimmune e.g. ITP
decreased production e.g. marrow failure

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6
Q

What are some causes of vascular disease resulting in failure of platelet plug?

A

hereditary e.g. Marfans

acquired e.g. old age, steroids, scurvy, vasculitis

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7
Q

What causes von willebrand deficiency?

A

usually hereditary - VWF deficiency

  • autosomal dominant
  • variable severity
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8
Q

What pattern of bleeding do you see with failure of platelet plug formation?

A

primary haemostasis bleeding pattern

  • mucosal bleeding
  • purpura
  • menorrhagia
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9
Q

What can cause failure of fibrin clot formation?

A

multiple clotting factor deficiencies

single clotting factor deficiencies e.g. haemophilia

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10
Q

What can cause multiple clotting factor deficiencies?

A

liver failure
vitamin K deficiency or warfarin therapy
DIC

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11
Q

What can cause vitamin K deficiency?

A

warfarin therapy
malabsorption
poor dietary intake
obstructive jaundice (as you need bile salts for absorption)

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12
Q

What is DIC?

A

disseminated intravascular coagulation

excessive and inappropriate activation of the haemostatic system

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13
Q

What can cause DIC?

A

sepsis
hypovolaemic shock
obstetric emergencies

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14
Q

Why do obstetric emergencies cause DIC?

A

placenta rich in TF

infarcts activate haemostasis dramatically

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15
Q

Why is there end organ failure in DIC?

A

microvascular thrombus formation

then more damage results in more activation of coagulation, resulting in more thrombus etc etc

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16
Q

How is DIC treated?

A

treat underlying cause
platelet and plasma transfusion
fibrinogen replacement

17
Q

Why are FDPs/D Dimers increased in DIC?

A

as there is also excess fibrinolysis

18
Q

Why does vitamin K deficiency cause multiple clotting factor deficiencies?

A

factors II, VII, IX and X are all carboxylated by vit K

19
Q

Why does liver failure cause multiple clotting factor deficiency?

A

as clotting factors are synthesised in the liver

20
Q

What is haemophilia?

A

x linked hereditary disorder resulting in single clotting factor deficiencies

21
Q

What are the PT and APTT results in haemophilia?

A

normal PT

prolonged APTT

22
Q

What is the bleeding pattern in haemophilia?

A

secondary haemostasis

bleeding from medium to large vessels

23
Q

What clotting factor is deficient in haemophilia A?

A

factor VIII

24
Q

What clotting factor is deficient in haemophilia B?

A

factor IX

25
Q

Which haemophilia is more common?

A

A

26
Q

What are the clinical features of haemophilia?

A

prolonged bleeding after surgery, dental procedures, invasive procedures
haemarthoses (bleeding into joints)
haematomas (bleeding into muscle)
recurrent soft tissue bleeds

27
Q

Why is the PT normal in haemophilia?

A

as they affect factors VIII and IX
these are involved in the intrinsic pathway
PT is extrinsic pathway