HAEM - Clotting and Bleeding Disorders Flashcards

(92 cards)

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
How long does Warfarin take to have an effect
up to 5 days
26
C/I Warfarin (4)
Peptic ulcer disease Bleeding disorders Severe HTN Pregnancy
27
Target INR - Warfarin for DVT/PE
2-3
28
Target INR - Warfarin for AF
2-3
29
Target INR - Warfarin for rec DVT/PE
3-4
30
Target INR - Warfarin for prosthetic heart valves
3-4
31
NOACs - Xa inhibtors (3)
The Xabans Apixaban Rivaroxaban Edoxaban
32
How are Xaban's reversed
Andexanet alfa
33
Mode of action dabigatran
Thrombin inhibitor
34
Reversal of dabigatran by:
Idarucizumab
35
Which NOACs are approved for the 2'' prevention in pt w/ AF
Dabigatran Apixaban Rivaroxaban
36
+ves of NOACs (4)
Rapid onset coag > Predictable pharmacokinetics Less interactions w/ foods/Dx Dont need reg coag monitoring
37
-ve NOACs
Partially cleared by kidney | Hence need does reduction in CKD
38
Overcoag w/ Warfarin Mx- INR 4.5-6:
Reduce dose or omit | Restart when INR <5
39
Overcoag w/ Warfarin Mx - INR 6-8
Stop | Restart when INR <5
40
Overcoag w/ Warfarin Mx - INR >8 + minor/no bleed
Stop Warfarin | + 0.5-2mg vit K PO
41
Overcoag w/ Warfarin Mx - INR >8 + major bleed
Stop warfarin | + 5-10mg Vit K IV + octaplex or FFP
42
Why is Vit K not sufficient Mx for Warfarin OD in a major bleed
Can take hours to work
43
Mode of action LMWH
Inactivates factor Xa
44
route LMWH
LC
45
UFH - route
IV SC
46
When to use UFH over LMWH
When high risk of bleed | or in severe CKD when LMWH = C/I
47
Mode of action UFH
Potentiates anti thrombin III
48
Monitoring aim UFH
APTT at 6hrs | 1.5-2.5
49
SE of heparins (4)
BLEED Heparin induced thrombocytopenia (HIT) OP - l term Hyperkalaemia
50
C/I heparins (4)
Bleeding disorders Platelets <60 x 10 9 Prev HIT Peptic ulcer
51
Mx overcoagulation by heparins (2)
Stop infusion | Give protamine sulphate
52
2 drugs used in thrombolysis
Streptokinase | Alteplase
53
What is streptokinase
Purified fraction of filtrate obtained from haemolytic streptococci
54
What does streptokinase do
Activates plasminogen --> plasmin
55
Why is streptokinase not good for repeated use?
= antigenic
56
Mechanism of action alteplase
TPA increases plasminogen activation + fibrinolysis
57
C/I of thrombolysis (7)
``` Active bleed Severe HTN (>200/120) Recent head trauma Recent surgery Pregnancy/recent delivery 10days Severe liver disease/oesophageal varices Prolonged/traumatic CRP ```
58
What is thrombophilia
Inherited/acquired coagulopathy predisposing to thrombosis, usually vv
59
E.g.s of inherited thrombophilias (3)
APC resistance/Factor V Leiden Antithrombin III deficiency Prothrombin gene mutation
60
E..g of acquired thrombophilia
APLS
61
Indications for screening for a thrombophilia (6)
``` aa thrombosis <50 vv thrombosis <40 w/ no RF Familial VTE Rec unexplained VTE Unusual site thrombosis (mesenteric/portal) Rec foetal loss ```
62
Ix suspected thrombophilia's (5)
``` FBC + clotting Fibrinogen [ ] +/- APC resistance test Lupus anticoag/anti-cardio-lipin ab Antithrombin + Protein C/S assays PCR ```
63
If APC resistance test is +ve, what is the next Ix
Factor V Leiden mutation PCR
64
What is DIC
Systemic activation of coagulation pathways --> extensive IV anticoagulation + fibrin clot development
65
What happens in DIC
Thrombotic occlusion of aa microvasculature Depletion of clotting factors + consumption platelets --> haemorrhage + progressing to organ failure
66
Causes of DIC (7)
``` Infection --> sepsis Trauma Malig (leukaemia, solid tumours) Obstetric complication Sever LF Tissue destruction Toxic/immunogenic stimuli ```
67
CF DIC (3)
Bruising XS bleeding from sites RF
68
Ix DIC (4)
Low platelets + fibrinogen Raised PT, APTT, bleeding time Raised D dimer Broken RBC/schistocytes on film
69
Tx DIC (3)
Tx cause agressive resus, replace platelets, FPP, fibrinogen (cryopreicpitate) Protein C
70
Most common blood group UK
O (47%)
71
Blood group O antigens + ab
Antigens - none | Ab - Anti-A + Anti-B
72
Blood group A - antigens and ab
Antigens - A | Ab - Anti-B
73
Blood group B - antigens and ab
Antigens - B | Ab - Anti-A
74
Blood group AB - antigens and ab
Antigens - A + B | Ab - none
75
What does group and save involve
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
how long does it take to do a cross match
40 mins
77
How long does it take to do a G+S
40 mins
78
In an emergency situation, how quickly can ABO matching be done (without antibody testing)
12 mins
79
What are packed red cells
All plasma is removed and replaced by additive --> in acute blood loss
80
What is FFP used for
Replacing coagulation factors in acquired deficiencies
81
What is cryoprecipitate useful for
DIC
82
How often should a transfusion reaction be monitored?
Ev 30 mins
83
Sx of acute haemolytic reaction during transfusion (5)
``` Agitation Raised T Low BP ABdo/Chx pain Signs DIC ```
84
Mx acute haemolytic reaction (4)
STOP transfusion A-E Keep IV line 0.9% saline
85
Mx of rash/urticaria during transfusion
Slow/stop transfusion + chloramphenamine
86
Mx of anaphylaxis during transfusion
STOP transfusion | Tx as per anaphylaxis guidelines
87
Mx of bacterial contamination of blood product for transfusion
Sepsis 6 protocol | Send blood unit to lab
88
What is TRALI
Transfusion related acute lung injury | ARDS b/c ab in donor plasma
89
PS TRALI (2)
Dyspnoea | CXR white out
90
Sx non-haemolytic ferbrile transfusion reaction
Shivering | Fever ½-1h after starting
91
Mx non-haemolytic febrile transfusion reaction (3)
Slow transfusion Give antipyretic Monitor
92
Mx fl overload during transfusion
Stop/slow Give O2 + diuretic Consider exchange transfusion