Bleeding disorders Flashcards

1
Q

After injury, what 3 processes halt bleeding?

A

Vasoconstriction
Gap plugging by platelets
Coagulation cascade

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What 3 groups do disorders of haemostasis fall into?

A

Vascular defects
Platelet defects
Coagulation defects

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What pattern of bleeding do coagulation disorders cause?

A

Delayed bleeding

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What pattern of bleeding do vascular and platelet disorders cause?

A

Prolonged bleeding

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Give some congenital examples of vascular defects

A

Osler-weber-rendu syndrome

Connective tissue disease (Ehlers danlos)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Give some acquired examples of vascular defects

A
Senile purpura
Infection - meningococcal, measles, dengue fever
Steroids
Scurvy (perifollicular haemorrhages) 
Henoch-schonlein purpura
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Give some examples of platelet disorders caused by decreased marrow production

A

Aplastic anaemia
Megaloblastic anaemia
Marrow infiltration (leukaemia and melanoma)
Marrow suppression (cytotoxic drugs and radiotherapy)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Give some examples of platelet disorders caused by increased destruction

A
Immune thrombocytopenia (ITP)
SLE
CLL
Drugs - heparin
Virus
DIC
Thrombotic thrombocytopenia (TTP)
HUS
Sequestration (in hypersplenism)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Give some platelet disorders caused by poorly functioning platelets

A

Myeloproliferative disease
NSAIDs
Increased urea

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Give some examples of congenital coagulation disorders

A

Haemophilia

Von Willebrand’s disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Give some examples of acquired coagulation disorders

A

Anticoagulants
Liver disease
DIC
Vitamin K defiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is haemophilia A a deficiency of?

A

Factor 8

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

How is haemophilia A inherited?

A

X-linked recessive condition, however 30% of cases arise from new mutations

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Describe the clinical presentation of haemophilia A

A

Depends on the severity
Often early in life or after surgery/trauma
Bleeds into joints - arthropathy
Bleeding into muscles - haematomas (increased pressure can lead to nerve palsies and compartment syndrome)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

How is haemophilia A diagnosed?

A

Increased APTT

Decreased factor 8 assay

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

How is haemophilia A managed?

A

Minor bleeding - pressure and elevation
Major bleeding - desmopressin - raises factor 8 - raised by 50% may be sufficient
Severe bleeding - recombinant factor 8 to raise the levels by 100%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What must be avoided in haemophilia A?

A

NSAIDs and IM injections

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is haemophilia B otherwise known as?

A

Christmas disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

How do you treat haemophilia B

A

Recombinant factor 9

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What is haemophilia B

A

Factor 9 deficiency

21
Q

What is acquired haemophilia caused by?

A

Suddenly appearing autoantibodies to factor 8

22
Q

What is the clinical presentation of acquired haemophilia?

A

Big mucosal bleeds in males and females

23
Q

How is acquired haemophilia diagnosed?

A

Increased APTT
Increased factor 8 autoantibody
Factor 8 activity <50%

24
Q

How do you treat acquired haemophilia

A

Steroids

25
Q

Describe the bleeding disorder produced from liver disease

A

Complicated
Decreased synthesis of clotting factors
Decreased absorption of vitamin K
Abnormalities of platelet function

26
Q

What bleeding disorder does malnutrition lead to?

A

Decreased vit K

Decreased production of clotting factors 2,7,9,10

27
Q

How do you treat bleeding due to decreased vit K absorption

A

Give 10mg IV vitK

In acute haemorrhage give FFP or human prothrombin complex

28
Q

Describe the process of fibrinolysis

A

Plasminogen is converted to plasmin by tissue plasminogen activator. Plasmin can then cleave fibrin and other factors. t-PA and plasminogen both bind to fibrin and thus localise the fibrinolysis to the area of the clot

29
Q

Name some fibrinolytic agents

A

Streptokinase
Alteplase
Tenecteplase
Reteplase

30
Q

Describe how streptokinase works

A

Streptococcal exotoxin that binds and activates plasminogen

31
Q

What is the risk of fibrinolytic agents?

A

Haemorrhage

32
Q

What is the risk of streptokinase?

A

Anaphylaxis on repeated doses

33
Q

Describe the pathophysiology of immune thrombocytopenia (ITP)

A

Caused by antiplatelet autoantibodies

34
Q

Describe the clinical presentation of immune thrombocytopenia (ITP)

A

Children - 2 weeks after infection, Sudden, self limiting purpura
It may also be chronic in women - purpura (pressure areas), menorrhagia, epistaxis, bleeding

35
Q

How is ITP diagnosed?

A

Increased megakaryocytes seen on marrow

Antiplatelet autoantibodies may be present

36
Q

How is ITP managed?

A

No treatment if mild
If symptomatic however or if platelets <20, give prednisolone. IV immunoglobulin may temporality rise platelets for surgery/pregnancy
If relapses - splenectomy or be cell depletion with rituximab
Eltromopag and romiplostim are alternative options for those with refractory disease

37
Q

What is osler-weber-rendu otherwise known as?

A

Hereditary haemorrhagic telangiectasia

38
Q

Describe hereditary haemorrhagic telangiectasia

A
Autosomal dominant
Multiple telangiectasia on skin and mucous membranes 
Epistaxis 
Telangiectasias at multiple sites
Visceral lesions - AVM
First degree family relative with HHT
39
Q

What is the most common inherited clotting disorder?

A

Von Willebrand

40
Q

What does the prothrombin time test?

A

The extrinsic pathway

Factors 1,2,5,7,10

41
Q

What causes the prothrombin time to be prolonged?

A

Warfarin
Vit K deficiency
Liver disease
DIC

42
Q

What does activated partial thromboplastin time (APTT) test?

A

The intrinsic pathway

Factors 1,2,5,8,9,10,11,12

43
Q

What prolongs the APPT?

A

Heparin treatment
Liver disease
DIC
Haemophilia

44
Q

What is the thrombin time?

A

Time taken for thrombin to convert fibrinogen to fibrin

45
Q

What prolongs the thrombin time

A

Heparin treatment
DIC
Dysfibrinogenaemia

46
Q

What are D-dimers?

A

Fibrin degradation products, released from cross linked fibrin during fibrinolysis

47
Q

When are D-dimers released?

A

DIC
VTE/PE
Inflammation - infection/malignancy

48
Q

What questions should you ask in a pre-op assessment to establish bleeding risk?

A

Previous history of any excessive bleeding
Drug history - anything which may affect haemostasis
PMH - lupus or liver disease