Bleeding Disorders Flashcards

1
Q

What can lead to failure of primary haemostasis

A

Vessel wall abnormalities - vasculitis or collagen disorders
Platelet abnormalities: reduced number (affects bone marrow), reduced function (immune thrombocytopaenia)
VWF: usually reduced amount of VWF but can also be due to abnormal VWF (hereditary)

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2
Q

What acquired vascular abnormalities can lead to bleeding disorders

A

Vasculitis - leads to leaky vessel walls

e.g. Henoch-schonlein purpura seen in kids after a viral infection

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3
Q

How does Henoch-schonlein purpura present

A

PR bleeding
Pupura on the lower limbs
Common in paeds
Seen after viral infection

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4
Q

When might you see scurvy

A

Alcoholics with a poor diet

ED

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5
Q

What can cause a thrombocytopaenia

A

Hereditary causes - rare
Marrow disorder - reduced production
Autoimmune conditions - increased platelet destruction

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6
Q

List causes of peripheral platelet destruction

A

DIC - used up
Autoimmune destruction - Immune thrombocytopenic purpura
Hypersplenism

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7
Q

What can cause functional deficits in platelets

A

Hereditary causes - very rare
Drugs most common (often deliberately – antiplatelets)
Renal failure - uraemia interferes with their function

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8
Q

What type of inheritance is vWF deficiency

A

Autosomal dominant

It is a common condition

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9
Q

What bleeding pattern does vWF deficiency have

A

Primary haemostatic problems such as mucosal bleeding – menorrhagia, nosebleeds, mouth

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10
Q

How can you treat peripheral platelet destruction due ot ITP

A

Suppress the antibody with steroids

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11
Q

How do you treat thrombocytopaenia caused by marrow failure

A

Platelet transfusion

It is a failure of production so would replace it

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12
Q

Are single clotting factor deficiencies usually hereditary or acquired

A

Usually hereditary

e.g. haemophilia

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13
Q

Are multiple clotting factor deficiencies usually hereditary or acquired

A

Generally acquired

e.g. DIC

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14
Q

What can lead to multiple factor deficiencies

A

Liver failure

Vitamin K deficiency or warfarin therapy

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15
Q

Where are clotting factors produced

A

All are made in the liver

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16
Q

Which clotting factors are dependent on vitamin K

A

2, 7, 9 and 10

17
Q

What can cause vitamin K deficiency

A
Poor dietary intake
Malabsorption
Obstructive jaundice
Vitamin K antagonists (warfarin)
Haemorrhagic disease of the newborn
18
Q

How does DIC present

A

End organ failure - caused by microvascular thrombi

Bruising, purpura and generalised bleeding - occur as the clotting factors are used up

19
Q

What can cause DIC

A

Sepsis
Obstetric emergencies
Malignancy
Hypovolaemic shock

20
Q

How do you treat DIC

A

Treat the underlying cause
Replacement therapy= Platelet transfusions
Plasma transfusions
Fibrinogen replacement

21
Q

What pattern of inheritance does haemophilia follow

A

X-linked
Therefore much more common in men a
Females are carriers

22
Q

Which type of haemophilia is more common

A

A

23
Q

What are the clinical features of severe haemophilia

A

Recurrent Hemarthroses - joint bleeds
If repeated can lead to arthritis and needs replacement
Recurrent soft tissue bleeds
Bruising in toddlers
Prolonged bleeding after dental extractions, surgery and invasive procedures

24
Q

What is the definition of massive haemorrhage by clinical situation

A

Bleeding that leads to a HR of over 110 and/or systolic of less than 90
Bleeding which has already prompted use of emergency O Rh(D) negative red cells

25
Q

What is the definition of massive haemorrhage by volume and rate

A

One blood volume in 24 hours - e.g. lose all blood
50% blood volume lost in 3 hours
Obviously cannot wait until its gets that bad
Blood loss of 150 ml/minute

26
Q

How do you manage a massive haemorrhage

A

Trigger the massive haemorrhage protocol
Stop the source of the bleeding
Supportive treatment with blood components

27
Q

What is the massive haemorrhage protocol

A

Activated by anyone for any patient - standard procedure for all
Allows emergency issue of blood components- always issued 4 units RBC, 4 units FFP and 1 unit platelets
Clinical staff take urgent blood samples and gain wide bore IV access
The blood bank will send up a pack ASAP

28
Q

What is the purpose of replacing red cells in massive haemorrhage

A

Maintains tissue oxygenation

29
Q

What is the purpose of giving FFP in massive haemorrhage

A

Replace coagulation factors and help maintain coagulation close to normal

30
Q

What is the purpose of giving cryoprecipitate in massive haemorrhage

A

Replace fibrinogen

31
Q

What is cryoprecipitate

A

It is processed from FFP and contains fibrinogen, factor 8 and VWF

32
Q

What is tranexamic acid

A

It is an anti-fibrinolytic which prevents fibrinolysis

Used in the treatment of major blood loss