Bleeding Disorders Flashcards

1
Q

what are causes of a failure of platelet plug formation

A

vascular
platelets
VWF

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2
Q

what are examples of vascular abnormalities that can cause problems

A

Marfan’s Syndrome
- have less collagen in their vessel walls

Vasculitis
- Henoch-Schonlein Purpura

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3
Q

what are acquired causes of thrombocytopenia

A
  1. reduced production
    - marrow problem [along with pancytopenia]
  2. increased destruction [if there was just a decrease in platelets]
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4
Q

what is thrombocytopenia

A

to few platelets

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5
Q

what are causes of platelet destruction

A

DIC = coag factors would fall too

Immune thrombocytopenic purpura (ITP) [autoimmune]

Hypersplenism = glandular fever, malignancy

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6
Q

what is the mechanism of destruction in ITP

A

antibodies stick to the platelet (IgG) which is then picked up by the spleen and removed

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7
Q

what drugs can cause an acquired platelet defect

A

aspirin

NSAID

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8
Q

apart from drugs, what else can cause an acquired platelet defect

A

renal failure

- urea builds up and effects the platelet function

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9
Q

what causes vWF deficiency - acquired

A

rare;
would be due to an antibody against vWF;
associated with hyperthyroidism

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10
Q

what causes vWF deficiency - hereditary

A

Autosomal dominant

reduction in vWF (more common) or reduced function in vWF (more severe)

Variable severity

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11
Q

what are causes of failure of a fibrin clot formation

A

DIC
Liver disease
[vitamin K deficiency leading to abnormal production of clotting factors]
Haemophilia

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12
Q

what tests results would suggest a multiple factor deficiency such as DIC or liver failure

A

prolonged PT AND APTT

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13
Q

where are all clotting factors made

A

the liver

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14
Q

what clotting factors require vitamin K

A

2 (prothrombin)
7
9
10

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15
Q

why do clotting factors need vitamin K

A

carboxylated by vitamin K = gives them a negative charge

w/out this they would not be attracted by the platelet plug to go to the area

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16
Q

what are sources of Vitamin K

A

diet - leafy green vegs [broccoli, sprouts]

17
Q

where is vitamin K absorbed

A

upper intestine

18
Q

what is required for vitamin K absorption

A

bile salts

- any gallstones in the gallbladder can lead to disruption

19
Q

what are causes of Vit K deficiency

A
Poor dietary intake
Malabsorption
Obstructive jaundice
Vitamin K antagonists (warfarin)
Haemorrhagic disease of the newborn
20
Q

what are babies given to avoid hemorrhagic disease of the newborn

A

IM Vit K injections

21
Q

what is the definition of Disseminated Intravascular Coagulation (DIC)

A

Excessive and inappropriate activation of ALL the haemostatic system

22
Q

what happens in DIC

A

Microvascular thrombus formation caused by platelet and fibrin clots
- causes end organ failure

THEN Clotting factor consumption as the body uses the factors up
- see bruising, purpura and generalised bleeding

23
Q

what causes DIC

A

mass release of tissue factor

- can be due to sepsis, tumour, obstetric problems, hypocolaemic shock

24
Q

what the lab findings in DIC

A

Prolonged PT
Prolonged APTT

Very High D-Dimer

25
Tx of DIC
treat the underlying cause replacement therapy - platelet transfusion - plasma transfusions - fibrinogen replacement
26
what is haemophilia
An X-linked, hereditary, life long disorder in which abnormally prolonged bleeding recurs episodically at one or a few sites on each occasion (called target joints)
27
what does X-linked mean
girls are carriers | boys are affected
28
what are the forms of haemophilia
Haemophilia A - factor 8 deficiency - most common Haemophilia B - factor 9 deficiency
29
what are common target joints
ankles knees elbows
30
what happens in target joins
bleed in, blood sits there, get iron, causing inflammation, new blood vessels form that are v fragile, can bleed again from these, more iron, more damage untreated can lead arthropathy
31
what is important to remember in haemophilia
no abnormality of primary haemostasis | i.e. something like a paper cut would heal fine
32
what lab results are seen in haemophilia
PT = NORMAL APTT = markedly PROLONGED
33
what are features of severe haemophilia
Recurrent Haemarthroses (ankle, knees, elbows) Recurrent soft tissue bleeds Prolonged bleeding after dental extractions, surgery and invasive procedures