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Haematology Year 3 > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

causes of failed platelet plug formation

A

vascular problem – decreased collagen
platelet problem – decreased numbers/function
VWF – decreased production/function

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2
Q

examples of vascular diseases that can cause failed platelet plug formation

A

marfans syndrome
– have less collagen in their vessel walls

vasculitis
– Henoch- Schonlein purpura

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3
Q

what is honoch schonlein purpura

A

IgA vasculitis
commonly occurs in children
causes vessels in the skin, joints, bowel and kidney to leak

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4
Q

what is thrombocytopenia

A

too few platelets

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5
Q

congenital cause of thrombocytopenia

A

Fanconi’s anaemia

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6
Q

which two mechanisms can lead to acquired thrombocytopenia

A
  1. reduced production

2. increased destruction

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7
Q

what causes acquired decreased production of platelets

A 
marrow failure
megaloblastic anaemia 
leukaemia 
myeloma 
myelofibrosis 
aplastic anaemia
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8
Q

what causes increased destruction of platelets

A

coagulopathy – disseminated intravascular coagulation

autoimmune – immune thrombocytopenic purpura (ITP)

hypersplenism

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9
Q

In children, what does immune thrombocytopenic purpura (ITP) often follow

A

viral infection

- rapid onset of purpura that is usually self limiting

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10
Q

who is ITP most commonly seen in

A

young women

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11
Q

features of ITP

A

often occurs with other autoimmune disorders e.g. SLE/thyroid disease

fluctuating course with easy bruising, epistaxis and menorrhagia

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12
Q

1st line treatment of ITP

A

oral corticosteroids

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13
Q

when might IV IgG be used in a patient with immune thrombocytopenic purpura

A

when a rapid rise in platelets is required e.g. before surgery

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14
Q

2nd line treatment of ITP

A

splenectomy

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15
Q

drugs that can cause platelet functional defects

A

aspirin

NSAIDS

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16
Q

how does renal failure cause an acquired platelet defect

A

urea builds up and effects platelet function

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17
Q

what causes an acquired VWF deficiency

A

antibody formation against VWF

  • rare
  • associated with hyperthyroidism
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18
Q

what causes a hereditary VWF deficiency

A

Type 1 - autosomal dominant, partial decrease in VWF
Type 2- abnormal VWF
Type 3- autosomal recessive, complete lack of VWF

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19
Q

what can cause failure of fibrin clot formation

A

multiple clotting factor deficiencies

  • Disseminated intravascular coagulation
  • vitamin K deficiency
  • liver disease
  • usually acquired

single clotting factor deficiency

  • haemophilia
  • usually hereditary
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20
Q

which clotting factors require vitamin K for formation

A

II (prothrombin), VII, IX, X

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21
Q

what is clotting factor II also know as

A

prothrombin

22
Q

where are all clotting factors made

A

the liver

23
Q

why do clotting factors need vitamin K

A

they are carboxylated by vitamin K – gives them a negative charge

without this they would not be attracted by the platelet plug

24
Q

what drug causes vitamin K deficiency

A

warfarin

25
Q

causes of vitamin K deficiency

A 
poor dietary intake 
malabsorption 
obstructive jaundice 
warfarin 
haemorrhage disease of the new born
26
Q

dietary sources of vitamin K

A

leafy green veg e.g broccoli, spinach, kale

27
Q

where is vitamin K absorbed

A

upper intestine

28
Q

what is required for vitamin K absorption

A

bile salts

- gallstones in the gallbladder can lead to disruption

29
Q

what are babies given to prevent haemorrhagic disease of the new born

A

IM Vit K

30
Q

what test results would suggest a multiple factor deficiency

A

prolonged PT + APTT

31
Q

what is disseminated intravascular coagulation

A

excessive + inappropriate activation of the haemostatic system
- primary, secondary + fibrinolysis

32
Q

what causes DIC

A 
mass release of tissue factor 
can be due to
- sepsis
- tumour 
- trauma
- surgery
- obstetric problems
- hypovolaemic shock
33
Q

effects of DIC

A

microvascular thrombus formation caused by platelet + fibrin clots
- causes end organ failure

clotting factor consumption as the body uses the factors up
- see bruising, purpura + generalised bleeding

secondary activation of fibrinolysis
- production of FDP

34
Q

lab findings in DIC

A

prolonged PT + APTT

high D dimer

35
Q

treatment of DIC

A

treat underlying cause

replacement therapy

  • platelet transfusion
  • fibrinogen replacement - cryoprecipitate
36
Q

what is haemophilia

A

X linked hereditary disorder in which abnormally prolonged bleeding recurs episodically at one or a few sites – called target joints

37
Q

how is haemophilia inherited

A

X linked

  • girls are carriers
  • boys are affected
38
Q

what are the forms of haemophilia

A

Haemophilia A

Haemophilia B

39
Q

what factor is deficient in haemophilia A

A

factor VIII

- most common

40
Q

what factor if deficient in haemophilia B

A

factor IX

41
Q

common target joints

A

ankles
elbows
knees

42
Q

what happens in target joints

A

bleeding, blood pools there, iron causes inflammation, new blood vessels form that are fragile
– can bleed again, more iron = more damage

untreated can lead to arthropathy

43
Q

features of haemophilia

A

recurrent haemathroses
recurrent soft tissue bleeds
prolonged bleeding after dental extractions, surgery and invasive procedures

44
Q

what is important to remember about haemophilia

A

no problems with primary haemostasis- only secondary is affected
therefore small bleeds like paper cuts would heel fine

45
Q

lab results in haemophilia

A

NORMAL PT time

APTT - markedly PROLONGED

46
Q

Treatment of haemophilia A

A

IV recombinant factor VIII

47
Q

what can be given in mild haemophilia A

A

desmopressin - raises factor VIII levels

48
Q

treatment of haemophilia B

A

IV facor IX

desmopressin is ineffective

49
Q

treatment of VWF disease

A

tranexamic acid

desmopressin

50
Q

who gets DIC

A

hospitalised patients with IV lines

Haematology Year 3 (21 decks)

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