HAEM - Clotting and Bleeding Disorders Flashcards

1
Q

What is Haemophilia A

A

Factor VIII deficiency

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2
Q

Inheritance Haemophilias

A

X linked recessive

+ high rates new mutations)

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3
Q

What is Haemophilia B

A

Factor IX deficiency

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4
Q

Which haemophilia is more common

A

A

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5
Q

Clinical features of haemophilias (3)

A

Major bleeds from minor trauma
Rec haemarthroses –> arthropathies
Compartment syndrome/nn palsies

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6
Q

Ix Haemophilias (2)

A

Raised APTT

+ Low VIII/IX on assays

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7
Q

Mx Haemopilias - general (2)

A

Avoid NSAIDs

Avoid IM injections

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8
Q

Mx minor bleed - haemophilias (2)

A

Compression + elevation

Desmopressin

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9
Q

Mx major bleed - haemophilias

A

Recombinant factor 8/9 to raise factors to 50% of normal

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10
Q

Mx life threatening bleed - haemophilia

A

Recombinant factor 8/9 to raise factors to 100% of normal

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11
Q

What is von willebrands disease

A

Abscence of vWF –> platelet type disorder

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12
Q

PS vWD

A

bleeding post op
Menorrhagia
Epistaxis

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13
Q

Difference between AD and AR vWD

A

AR - complete loss vWF (20%)

AD = less severe depletion (80%)

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14
Q

Ix vWD (2)

A

APTT increase

INR/platelets within normal limit

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15
Q

Mx vWD

A

Expert Mx
Transenamic acid for minor bleed
Desmopressin/recom factor 8 if more severe)

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16
Q

Site of bleed + clue it gives for condition - mm/joint

A

Coagulation issue

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17
Q

Site of bleed + clue it gives for condition - purpura/epistaxis/menorrhagia/GI haemorrhage

A

Platelet issue/vWd

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18
Q

Site of bleed + clue it gives for condition - rec bleeds @ 1 site

A

Local endothelial abnormality

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19
Q

What does bleeding immediately after surgery tell you

A

= Platelet issue

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20
Q

What does bleeding several hours after surgery tell you

A

= coagulation issue

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21
Q

Clopidogrel mode of action

A

Irreversible modification of platelet receptor

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22
Q

Dipyrimadole

A

PDE inhibitor (affects aggregation)

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23
Q

Mode of action Heparin

A

Activates AT3 –> inactivation of thrombin + X

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24
Q

Desmopression mode of action

A

Stimulates exocytosis of vWF

Hence is useful in vWD/Haemophilia A

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25
Q

How long does Warfarin take to have an effect

A

up to 5 days

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26
Q

C/I Warfarin (4)

A

Peptic ulcer disease
Bleeding disorders
Severe HTN
Pregnancy

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27
Q

Target INR - Warfarin for DVT/PE

A

2-3

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28
Q

Target INR - Warfarin for AF

A

2-3

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29
Q

Target INR - Warfarin for rec DVT/PE

A

3-4

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30
Q

Target INR - Warfarin for prosthetic heart valves

A

3-4

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31
Q

NOACs - Xa inhibtors (3)

A

The Xabans
Apixaban
Rivaroxaban
Edoxaban

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32
Q

How are Xaban’s reversed

A

Andexanet alfa

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33
Q

Mode of action dabigatran

A

Thrombin inhibitor

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34
Q

Reversal of dabigatran by:

A

Idarucizumab

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35
Q

Which NOACs are approved for the 2’’ prevention in pt w/ AF

A

Dabigatran
Apixaban
Rivaroxaban

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36
Q

+ves of NOACs (4)

A

Rapid onset coag
> Predictable pharmacokinetics
Less interactions w/ foods/Dx
Dont need reg coag monitoring

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37
Q

-ve NOACs

A

Partially cleared by kidney

Hence need does reduction in CKD

38
Q

Overcoag w/ Warfarin Mx- INR 4.5-6:

A

Reduce dose or omit

Restart when INR <5

39
Q

Overcoag w/ Warfarin Mx - INR 6-8

A

Stop

Restart when INR <5

40
Q

Overcoag w/ Warfarin Mx - INR >8 + minor/no bleed

A

Stop Warfarin

+ 0.5-2mg vit K PO

41
Q

Overcoag w/ Warfarin Mx - INR >8 + major bleed

A

Stop warfarin

+ 5-10mg Vit K IV + octaplex or FFP

42
Q

Why is Vit K not sufficient Mx for Warfarin OD in a major bleed

A

Can take hours to work

43
Q

Mode of action LMWH

A

Inactivates factor Xa

44
Q

route LMWH

A

LC

45
Q

UFH - route

A

IV SC

46
Q

When to use UFH over LMWH

A

When high risk of bleed

or in severe CKD when LMWH = C/I

47
Q

Mode of action UFH

A

Potentiates anti thrombin III

48
Q

Monitoring aim UFH

A

APTT at 6hrs

1.5-2.5

49
Q

SE of heparins (4)

A

BLEED
Heparin induced thrombocytopenia (HIT)
OP - l term
Hyperkalaemia

50
Q

C/I heparins (4)

A

Bleeding disorders
Platelets <60 x 10 9
Prev HIT
Peptic ulcer

51
Q

Mx overcoagulation by heparins (2)

A

Stop infusion

Give protamine sulphate

52
Q

2 drugs used in thrombolysis

A

Streptokinase

Alteplase

53
Q

What is streptokinase

A

Purified fraction of filtrate obtained from haemolytic streptococci

54
Q

What does streptokinase do

A

Activates plasminogen –> plasmin

55
Q

Why is streptokinase not good for repeated use?

A

= antigenic

56
Q

Mechanism of action alteplase

A

TPA increases plasminogen activation + fibrinolysis

57
Q

C/I of thrombolysis (7)

A
Active bleed 
Severe HTN (>200/120) 
Recent head trauma 
Recent surgery
Pregnancy/recent delivery 10days 
Severe liver disease/oesophageal varices
Prolonged/traumatic CRP
58
Q

What is thrombophilia

A

Inherited/acquired coagulopathy predisposing to thrombosis, usually vv

59
Q

E.g.s of inherited thrombophilias (3)

A

APC resistance/Factor V Leiden
Antithrombin III deficiency
Prothrombin gene mutation

60
Q

E..g of acquired thrombophilia

A

APLS

61
Q

Indications for screening for a thrombophilia (6)

A
aa thrombosis <50 
vv thrombosis <40 w/ no RF
Familial VTE
Rec unexplained VTE 
Unusual site thrombosis (mesenteric/portal) 
Rec foetal loss
62
Q

Ix suspected thrombophilia’s (5)

A
FBC + clotting
Fibrinogen [ ] +/- APC resistance test
Lupus anticoag/anti-cardio-lipin ab 
Antithrombin + Protein C/S assays
PCR
63
Q

If APC resistance test is +ve, what is the next Ix

A

Factor V Leiden mutation PCR

64
Q

What is DIC

A

Systemic activation of coagulation pathways –> extensive IV anticoagulation + fibrin clot development

65
Q

What happens in DIC

A

Thrombotic occlusion of aa microvasculature
Depletion of clotting factors + consumption platelets –> haemorrhage
+ progressing to organ failure

66
Q

Causes of DIC (7)

A
Infection --> sepsis 
Trauma 
Malig (leukaemia, solid tumours) 
Obstetric complication 
Sever LF
Tissue destruction 
Toxic/immunogenic stimuli
67
Q

CF DIC (3)

A

Bruising
XS bleeding from sites
RF

68
Q

Ix DIC (4)

A

Low platelets + fibrinogen
Raised PT, APTT, bleeding time
Raised D dimer
Broken RBC/schistocytes on film

69
Q

Tx DIC (3)

A

Tx cause
agressive resus, replace platelets, FPP, fibrinogen (cryopreicpitate)
Protein C

70
Q

Most common blood group UK

A

O (47%)

71
Q

Blood group O antigens + ab

A

Antigens - none

Ab - Anti-A + Anti-B

72
Q

Blood group A - antigens and ab

A

Antigens - A

Ab - Anti-B

73
Q

Blood group B - antigens and ab

A

Antigens - B

Ab - Anti-A

74
Q

Blood group AB - antigens and ab

A

Antigens - A + B

Ab - none

75
Q

What does group and save involve

A

Taking a sample from pt and determining what ABO group the pt blood is + screening for ab that may cause haemolysis reaction in transfused blood

76
Q

how long does it take to do a cross match

A

40 mins

77
Q

How long does it take to do a G+S

A

40 mins

78
Q

In an emergency situation, how quickly can ABO matching be done (without antibody testing)

A

12 mins

79
Q

What are packed red cells

A

All plasma is removed and replaced by additive –> in acute blood loss

80
Q

What is FFP used for

A

Replacing coagulation factors in acquired deficiencies

81
Q

What is cryoprecipitate useful for

A

DIC

82
Q

How often should a transfusion reaction be monitored?

A

Ev 30 mins

83
Q

Sx of acute haemolytic reaction during transfusion (5)

A
Agitation 
Raised T
Low BP 
ABdo/Chx pain 
Signs DIC
84
Q

Mx acute haemolytic reaction (4)

A

STOP transfusion
A-E
Keep IV line
0.9% saline

85
Q

Mx of rash/urticaria during transfusion

A

Slow/stop transfusion + chloramphenamine

86
Q

Mx of anaphylaxis during transfusion

A

STOP transfusion

Tx as per anaphylaxis guidelines

87
Q

Mx of bacterial contamination of blood product for transfusion

A

Sepsis 6 protocol

Send blood unit to lab

88
Q

What is TRALI

A

Transfusion related acute lung injury

ARDS b/c ab in donor plasma

89
Q

PS TRALI (2)

A

Dyspnoea

CXR white out

90
Q

Sx non-haemolytic ferbrile transfusion reaction

A

Shivering

Fever ½-1h after starting

91
Q

Mx non-haemolytic febrile transfusion reaction (3)

A

Slow transfusion
Give antipyretic
Monitor

92
Q

Mx fl overload during transfusion

A

Stop/slow
Give O2 + diuretic
Consider exchange transfusion