6. Disorders of haemostasis Flashcards

1
Q

what 2 things cause a bleeding disorder?

A

CF deficiency or dysfunctional CF

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2
Q

2 types of bleeding disorder

A

hereditary or acquired

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3
Q

4 types of hereditary bleeding disorder

A

Haemophilia A, B, C and vW disease

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4
Q

2 types of acquired bleeding disorders

A

consumption or production problem

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5
Q

bleeding disorder which is caused by a consumption problem

A

Disseminated IV coagulation

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6
Q

2 production problems in acquired bleeding disorder

A

Vit K def, Liver disease

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7
Q

8 main sxs of bleeding disorders

A
bleeding gums
purpura
chronic anaemia 
mennorhagia 
bruising 
petichae rash
joint bleeds 
muscle bleeds
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8
Q

what is Haemophilia A?

A

FVIII deficiency

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9
Q

what is Haemophilia B?

A

FIX deficiency

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10
Q

what is Haemophilia C?

A

FXI deficiency

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11
Q

5 CF examples which are autosomal recessive

A
prothrombin = IIa
fibrinogen = I 
FV
FVII
FXIII
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12
Q

where are autosomal reccessive CF disorders common?

A

countries with incest

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13
Q

3 causes of vitamin K deficiency

A
  1. inadequate diet
  2. malabsorption
  3. drugs (warfarin)
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14
Q

how does vitamin K deficiency affect haemostasis?

A

leads to decreased synthesis of F II, VII, IX, X

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15
Q

what is the APTT and PT in vit K def? why?

A

prolonged in both - as many factors involved which are part of instrinsic and extrinsic

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16
Q

what does liver disease cause?

A

impaired absorption of Vit K

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17
Q

how does liver diease affect haemostasis?

A

same effects of Vit K deficiency - leads to decreased synthesis of F II, VII, IX, X

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18
Q

what is desseminated intravascular coagulation?

A

blood clots block small vessels

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19
Q

how does DIC affect haemostasis?

A

excessive activation of tissue factor - therefore excessive coagulation

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20
Q

why can DIC lead to amputation?

A

deposition of fibrin in microcirculation

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21
Q

what is acquired haemophilia

A

inhibits factor VIII

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22
Q

who is acquired haemophilia prevalent in?

A

elderly pt with underlying malignant disease

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23
Q

2 types of platelet disorders

A

hereditary or acquired

24
Q

3 hereditary platelet disorders

A
  1. glanzmann’s thromboasthenia
  2. Bernard Soulier syndrome
  3. vW disease
25
Q

3 acquired platelet disorder types

A
  1. dysfuntional
  2. shortened lifespan
  3. production problems
26
Q

what can cause dysfunctional acquired platelet disorders?

A

drugs

27
Q

what can cause shortened lifespan platelet disorders?

A

malaria, bleeding

28
Q

what can cause platelet production disorders?

A

leukaemia
post-viral infection
immunosuppression

29
Q

examples of drugs which affect platelet function (10) 4 are not drugs

A
  1. caffeine
  2. NSAIDS/aspirin
  3. CV
  4. antimicrobials
  5. anti-coagulants
  6. chemotherapy drugs
  7. onions/garlic
  8. Vit E
  9. alcohol
  10. fish oil
30
Q

2 types of thrombotic disorders

A

thrombosis and hereditary thrombophilia

31
Q

2 types of thrombosis

A

arterial and venous

32
Q

4 examples of hereditary thrombophilia

A
  1. FV leiden mutation
  2. anti-thrombin deficiency
  3. protein S and C deficiency
  4. prothrombin gene mutation
33
Q

what is thrombosis?

A

formation of a solid mass (thrombus), in the lumen of a blood vessel

34
Q

what is a thrombus formed from

A

platelets and fibrin

35
Q

what 4 diseases are associated with thrombi?

A
  1. MI
  2. cerebral vascular disease
  3. Deep vein thrombosis
  4. pulmonary embolism
36
Q

describe pathogenesis of arterial thrombosis

A
  1. atherosclerosis
  2. injury to vessel
  3. blood leaks out and comes into contact with subendothelial collagen and TF
  4. primary haemostasis - formation of platelet plug
  5. thrombus blocks artery
37
Q

6 risk factors for arterial thrombosis

A
  1. diabetes
  2. smoking
  3. raised BP
  4. raised lipids
  5. male
  6. family history
38
Q

pathogenesis of venous thrombosis

A
  1. increased coagubility of blood
  2. blood flow slows
  3. coagulation at site of initiation of thrombus
39
Q

4 main risk factoes for venous thrombosis

A
  1. FV leiden gene mutation
  2. Blood group
  3. prothombin gene mutation
  4. protein S and C deficiency
40
Q

which type of thrombosis is caused by immobility?

A

venous

41
Q

what is the FV leiden mutation?

A

Arg replaced with glut - pos 560 on FV gene

42
Q

is antithrombin def autosomal dominant or recessive?

A

dominant

43
Q

how to treat antithrombin def?

A

anti-thrombin deficiency

44
Q

what are the effcts of prothrombin gene mutation?

A

increased levels of circualting prothrombin

45
Q

what is the prothrombin gene mutation?

A

Guan replaced by adenine - pos 20210

46
Q

role of protein S

A

co-factor to protein C

47
Q

is protien S def autosomal dominant or recessive?

A

dominant

48
Q

normal role of protein C

A

activated by thrombin

cleaves FVa and FVIII to inactivate them - prevents excessive clotting

49
Q

5 ways of treating thrombosis

A
  1. heparin
  2. warfarin
  3. FXa inhibitors
  4. direct thombin inhibitors
  5. anti-platelet drugs
50
Q

how is heparin given

A

IV

51
Q

how is warfarin given

A

orally

52
Q

give an example of FXa inihibitor

A

rivaroxaban

53
Q

which direct thrombin inhibitor replaces heparin?

A

Bivalirudin

54
Q

which direct thrombin inhibitor replaces warfarin?

A

dabigatran

55
Q

give an example of an antiplatelet drug

A

aspirin