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

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
What is the definition of massive haemorrhage by volume and rate
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
How do you manage a massive haemorrhage
Trigger the massive haemorrhage protocol Stop the source of the bleeding Supportive treatment with blood components
27
What is the massive haemorrhage protocol
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
What is the purpose of replacing red cells in massive haemorrhage
Maintains tissue oxygenation
29
What is the purpose of giving FFP in massive haemorrhage
Replace coagulation factors and help maintain coagulation close to normal
30
What is the purpose of giving cryoprecipitate in massive haemorrhage
Replace fibrinogen
31
What is cryoprecipitate
It is processed from FFP and contains fibrinogen, factor 8 and VWF
32
What is tranexamic acid
It is an anti-fibrinolytic which prevents fibrinolysis | Used in the treatment of major blood loss