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Haematology > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

Define bleeding diathesis

A

Increased susceptibility to bleeding due to qualitative/quantitative defects of inhibition of the function of platelets, vWF or coagulation factors

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2
Q

What are the key questions in a bleeding history?

A

Has patient got a bleeding disorder?
How severe is it?
Pattern of bleeding - thrombocytopenic/vWF vs coagulation
Congenital vs acquired - prev. episode/age of onset
FH

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3
Q

Where do the bleeds occur in mucosal (thrombocytopenic/low vWF) bleeding?

A

Mucosal, GI, menorrhagia, petechiae/purpura, epistaxis

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4
Q

Where do bleeds occur in coagulation occur deficiencies?

A

Articular, muscle haematomas, CNS

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5
Q

What is a classical presentation for someone with haemophilia?

A

Young boy presenting with intra-articular bleeds (esp. knee, ankle, shoulder, elbow)

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6
Q

What is key about the petechiae in thrombocytopenic bleeding?

A

Non-blanching

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7
Q

Why do you get muscle wasting in haemophilia?

A

Disuse

Muscle haematomas

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8
Q

How would a boy with haemophilia present with an intracranial bleed?

A

Focal signs, headache, loss of consciousness

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9
Q

What should you ask in history of bleeding about previous bleeds?

A 
Bruising 
Epistaxis
Post-surgical/trauma - tonsillectomy, dental surgery, circumcision, 
appendectomy
Menorrhagia 
PPH 
Post-trauma

HOW appropriate was the bleeding?

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10
Q

How is haemophilia inherited?

A

X-linked

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11
Q

How do the presentations of haemophilia A and B differ?

A

They don’t

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12
Q

What changes the severity of bleeding in haemophilia A and B?

A

The residual factor 8/9 in their circulation
<1% severe - unprovoked bleeding
1-5% moderate
5-30% - tend to be okay apart from maybe needing pre-surgical preventative measures

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13
Q

What is haemophilia A a deficiency of?

A

Factor 8

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14
Q

What is haemophilia B a deficiency of?

A

Factor 9

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15
Q

What are the clinical features of haemophilia?

A

Intra-articular bleeds (esp. into weight bearing, hinge joints)
Muscle haematomas (esp. calves and biceps)
CNS bleeding
Retroperitoneal bleeding
Post-surgical bleeding

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16
Q

What are the clinical complications of haemophilia?

A

Synovitis
Chronic haemophilic arthropathy
Neurovascular compression
Other sequlae of bleeding (stroke)

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17
Q

Why does chronic haemophilic arthropathy occur?

A

Macrophages eat blood in IA bleeds –> inflammatory cytokines released which drives degeneration of articular cartilage and joint –> end stage joint dx in young adulthood

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18
Q

What X-Ray findings will you get with end stage chronic haemophilic arthropathy?

A

Joint space narrowing

Development of osteophytes

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19
Q

How do you manage chronic haemophilic arthropathy?

A

Joint replacement

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20
Q

How do you diagnose haemophilia?

A

Typical history
Screening - APTT normal, AP normal (only screens for factors, 1, 2, 5, 7, 10)
Reduced factor 8/9 - check via assay
Genetic analysis to identify mutation

21
Q

How do you treat haemophilia?

A

Recombinant factor 8/9 (3x week for 8, 2wkly for 9)
DDVAP if mild
Tranexamic acid

Also may require splints, physio, analgesia, synovectomy, joint replacement

22
Q

How does DDVAP work?

A

Releases stored factor 8 and vWF from endothelium

23
Q

In which groups of people can you not use DDVAP for treatment of haemophilia?

A

Elderly and young kids as if may put them at risk of hyponatraemia

24
Q

What are the treatment complications of haemophilia?

A

HIV/HBV, HCV/vCJD transmission is kind of a thing of the past
Inhibitors - antibodies against factor 8 in nulmutations (as body not seen factor 8 before, don’t tend to get nulmutations in factor 9)

25
Q

How common is vWF disease?

A

1 in 200

26
Q

How many types are there?

A

3
First 2 AD
Type 3 is AR

27
Q

What are the clinical features of vWF disease?

A

Mucosal bleeding - post-surgical, GI, menorrhagia, petechiae/skin, epistaxis

28
Q

What is the deficit in type 1 vWF disease?

A

Quantitative deficiency

29
Q

What is the deficit in type 2 vWF disease?

A

Qualitative defect

30
Q

What is the deficit in type 3 vWF disease?

A

Complete, severe deficiency

31
Q

How do you treat vWF disease?

A 
vEF concentrate
DDAVP
Tranexamic acid
Topical applications
OCP
32
Q

What are the acquired bleeding disorders?

A 
Thrombocytopenia
Liver failure
Renal failure 
DIC 
Drugs (e.g. warfarin, clopidogrel, rivaroxaban, heparin, aspirin etc.)
33
Q

What are the reasons for thrombocytopenia?

A

Decreased production - marrow failure, asplasia, infiltration (e.g. ALL)
Increased consumption - immune ITP, DIC, hypersplenism (platelets caught in spleen)

34
Q

What is DIC?

A

Abnormal coagulation disorder that occurs in the context of burns, sepsis, eclampsia, malignancy etc.

Leads to widespread activation of coagulation –> microvascular thrombi –> organ ischaemia and failure & consumption of platelets –> bleeding elsewhere

35
Q

What is the lifespan of platelets?

A

10 days

36
Q

What are the clinical features of thrombocytopenia?

A

Petechiae (esp in legs)
Ecchymosis
Mucosal bleeding
Rarely CNS bleeding

37
Q

What is ITP?

A

Idiopathic thrombocytopenic purpura

Low platelets in absence of bone marrow failure and no other known cause
Tends to be autoimmune

38
Q

What are the two different presentations of ITP?

A

Acute in children

Chronic in adults

39
Q

What things are associated with ITP?

A

Infections (EBV, HIV)
Collagenosis
Lymphoma
Drug induced (quinine)

40
Q

How do you diagnose ITP?

A

Isolated thrombocytopenia
Blood film is fine
Tend not to need to check bone marrow

41
Q

How do you manage ITP?

A

Acute - rescue therapy with steroids and IV Ig

Chronic - splenectomy, thrombopoetin analogues (eltrombopag)

42
Q

How does liver failure affect clotting?

A

Rebalanced haemostasis –> less stable balance between coagulation and anti-coagulation as liver responsible for making factors contributing to both

43
Q

What factors are produced by the liver?

A

1, 2, 5, 7, 8, 9, 10, 11

44
Q

What APTT, PT and fibrinogen test results would you expect to see in liver failure?

A

Prolonged APTT, PT and reduced fibrinogen

45
Q

When do patients with liver failure tend to be at the biggest risk of bleeding?

A

From structural abnormalities, e.g. varices after trigger (e.g. infection)

46
Q

What are the vitamin K dependent factors?

A

2, 7, 9, 10

47
Q

How can you help prevent bleeds in patients with liver failure?

A

Vit K

FFP

48
Q

What is haemorrhagic disease of the newborn?

A

Newborn’s have an immature coagulation system and are deficient in vitamin K therefore are prone to fatal haemorrhage

49
Q

How is haemorrhagic disease of the newborn completely prevented?

A

Vit K injection at time of birth

Haematology (14 decks)

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