Bleeding Disorders Flashcards

1
Q

What is arterial thrombosis

A

When a blod clot forms in the arterial system

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2
Q

Risk factors for arterial thrombosis

A
Increasing age 
Smoking 
Sedentary lifestyle 
Hypertension 
DM 
Obesity 
Hypercholesterolemia
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3
Q

Describe the clot in arterial thrombosis

A

Platelets and fibrin

White clot

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4
Q

Pathology of arterial thrombosis

A

Results in infarction and ischaemia

Decrease in O2 supply

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5
Q

Example of arterial thrombosis

A

Coronary thrombosis disease:
MI
Angina

Cerebrovascular disease:
Stroke
TIA

Peripheral Disease:
Acute limb ischaemia
Chronic limb ischaemia

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6
Q

Acute presentation Rx for arterial thrombosis

A

Thrombolysis
Anti-platelet/anti-coagulant therapy
Interventional techniques e.g PCI

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7
Q

Primary Rx for arterial thrombosis

A

Lifestyle modification
Smoking cessation
Exercise
Diet ect..

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8
Q

What is a venous thrombosis

A

Thrombus arising in the venous system

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9
Q

Risk factors for venous thrombosis

A
Increasing age 
Pregnancy
Hormonal therapy (COCP/HRT)
Depoprovera (injection) higher risk than the other progesterone only methods) 
Tissue trauma 
Immobility 
Surgery 
Obesity 
Systemic disease
FH (mainly related to 1st degree relatives
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10
Q

Describe the clot in venous thrombosis

A

Fibrin and red cells

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11
Q

Which aspects of Virchow’s triad tend to be most affected in venous disease

A

Blood flow

Blood constituents

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12
Q

What are the 2 main clinical manifestations of venous thrombosis disease

A

Limb DVT

PE

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13
Q

Rarer clinical manifestations of venous thrombosis disease

A

Visceral venous thrombosis
Intracranial venous thrombosis
Superficial thrombophlebitis

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14
Q

Which Pre-test scores can be used in venous thrombosis disease

A

Wells Score

Geneva Score

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15
Q

If probability score is low what is the next Ix

A

D-dimer

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16
Q

If probability score is high what is the next Ix

A

D-dimer skipped

Go straight to imaging

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17
Q

What is D-dimer a breakdown product of?

A

Fibrinolysis

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18
Q

Imaging Ix for venous thrombosis

A

Doppler USS
V/Q scan
CT Pulmonary Angiogram

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19
Q

Drug Rx for venous thrombosis

A

Anti-coagulants
LMWH
Warfarin
DOACs

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20
Q

Example of case where thrombolysis would be used

A

Massive PE

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21
Q

What is heritable thrombophilia

A

Inherited predisposition to venous thrombosis

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22
Q

Most common type of heritable thrombophilia

A

Factor V Leiden

Prothrombin G20210A

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23
Q

Rare types of heritable thrombophilia

A

Anti-thrombin deficiency
Protein C deficiency
Protein S deficiency

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24
Q

Common type of Acquired haemophilia

A

Anti-phospholipid syndrome

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25
Q

What conditions are most associated with heritable thrombophilia

A

DVT

PE

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26
Q

Ix for thrombophilia

A

FBC
Blood film

Clotting:
PT 
Thrombin time 
APTT 
Fibrinogen
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27
Q

Rx for thrombophilia

A

No specific Rx

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28
Q

Who is thrombophilia prophylaxis not required for

A

Absence of VTE

But advise about risk with COCP or HRT

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29
Q

Who is prophylaxis required for in thrombophilia

A

Long term anticoagulation in those with recurrent episodes with no other risk factors

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30
Q

Who should be tested for thrombophilia

A
Arterial thrombus or MI <50yrs Unprovoked VTE <40yrs with no risk factors 
VTE with oral contraceptive/pregnancy
Unusual site thrombosis e.g mesenteric 
Recurrent fetal loss
Neonatal thrombosis
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31
Q

Pathology of prothrombin G20210A

A

Produce too much prothrombin
Increased tendency to clot
Increased risk of thrombosis

32
Q

Pathology of anti-thrombin defiency

A

Deficit in anti-thrombin

Anti-thrombin is a naturally occurring anti-coagulant

33
Q

Pathology of Protein C deficiency

A

Protein C is naturally occurring anti-coagulant

=> deficiency leads to increased risk of clots

34
Q

What is protein S

A

Naturally occurring anti-coagulant

35
Q

What does DIC stand for

A

Diffuse systemic coagulation activation

36
Q

When can DIC typically occur

A

Septicaemia
Malignancy
Eclampsia
Severe burns

37
Q

Pathology of DIC

A

Platelet and/or fibrin

Results in diffuse ischaemia affecting extremities

38
Q

Rx for DIC

A

Mainly directed towards underlying conditions

39
Q

Pathology of Factor V Leiden Syndrome

A

Hypercoagulable state
Prone to thrombosis

Mutated factor 5 is resistant to action of Protein C
Protein C is a natural anti-coagulant

Increased risk of developing DVT (PE) at some point

40
Q

Rx for Factor V Leiden Syndrome

A

No cure

Prevention of thrombotic complications using medication

41
Q

Miscarriage risk in Factor V Leiden Syndrome

A

Women with Factor V Leiden 2-3x risk of miscarriage

42
Q

What is deficient in Haemophilia A

A

Factor 8

43
Q

What is deficient in Haemophilia B

A

Factor 9

44
Q

Inheritance pattern Haemophili

A

X-linked

45
Q

What does the severity of bleeding in haemophilia depend on

A

Residual coagulation factor activity

46
Q

What is common in haemophilia with factor level <1%

A

severe

Common to have spontaneous bleeding

47
Q

Clinical features of haemophilia

A
Haemoarthrosis 
Muscle haematoma 
Post surgical bleeding 
Typically M 
6mnth to 1yr
48
Q

Which gender is affected by haemophilia

A

M

49
Q

Ix for haemophilia

A

Clinical
Prolonged APTT
Normal PT
Normal BT

Reduced
Factor 8 or 9 depending

50
Q

Rx for haemophilia

A

F VIII
F IX
Injections

51
Q

Prevention Rx for haemophilia

A
Aim to prevent spontaneous bleeding
Splints 
Physio.
Analgesia
Synovectomy 
Joint replacement
52
Q

Complications of bleeding into muscles

A

Increased pressure
can cause:
Nerve palsies
Compartment syndrome

53
Q

What blood cell does Von Willebrand Disease affect

A

Platelets

54
Q

What is the commonest inheritable bleeding disorder

A

Von Willebrand Disease

55
Q

Inheritance pattern of Von-Willebrand disease

A

Autosomal dominant

56
Q

What pattern of bleeding is seen in Von-Willebrand disease

A

Mucosal pattern of bleeding

57
Q

What are the 3 types of Von-Willebrand Disease

A

Type I, II, III

58
Q

Describe Type I Von Willebrand disease

A

Mildest from
Quantitative defect
Don’t make enough

59
Q

Describe Type II Von Willebrand Disease

A

Qualitative deficiency
Quality of the protein is not good enough
Doesn’t work properly

60
Q

Describe Type III Von Willebrand disease

A

Severe (complete deficiency)
Most severe
Rarest

61
Q

Clinical features of Von willebrand isease

A
Mucosal pattern of bleeding
Easily bruising 
Large bruises
Frequent/prolonged epistaxis
Bleeding gums 
Heavy/prolonged bleeding from cuts
Menorrhagia 
PPH (obs)
62
Q

Rx for Von Willebrand disease

A
wVF concenrate 
DDAVP 
Tranexamic acid 
Topical applicaitons 
OCP
63
Q

What is thrombocytopenia

A

Low platelet count

64
Q

what are the causes of thrombocytopenia

A

Decreased production

Increased consumption

65
Q

Decreased production causes of thrombocytopenia

A

Marrow failure
Aplasia
Infiltration

66
Q

Increased consumption causes of thrombocytopenia

A

Immune ITP
Non-immune DIC
Hypersplenism

67
Q

Clinical features of thrombocytopenia

A

Petechia
Ecchymosis
Mucosal pattern bleeding
Rare CNS bleeding

68
Q

What is Immune ITP

A

Defined as low platelet count with normal bone marrow

And absence of other causes of thrombocytopenia

69
Q

What causes immune ITP

A

Anti-platelet antibodies

Autoimmune

70
Q

Associations of Immune ITP

A

Infection esp. EBV, HIV
Colagenosis
Lymphoma
Drug induced (quinine esp.)

71
Q

2 distinct patterns of Immune ITP

A

Actue

Chronic

72
Q

Typical picture of acute immune ITP

A

Children
Following acute infection
Self-limiting purpura

73
Q

Typical picture of chronic immune ITP

A
Adults
Mainly women 
Fluctuating course 
Bleeding 
Epistaxis 
Menorrhagia
74
Q

Clinical picture of immune ITP

A
Increased tendency to bleed 
Petechiae 
Purpura 
Menorrhagia 
Epistaxis 
Bleeding from gums
75
Q

Ix for Immune ITP

A

Isolated thrombocytopenia

Only abnormality = > easy to Dx

Diagnosis of exclusion

Bone marrow?
Very rarely need to do tis to Dx

76
Q

Rx for Immune ITP

A

Steroids:
Dexamethasone
Prednisolone

IgG transfusion

Thrombopoietin analogous:
Romiplostim

Oral thrombopoietin receptor agonist:
Eltrombopag

77
Q

What is Immune ITP a Dx of

A

Dx of exclusion