Bleeding Disorders Flashcards

1
Q

Normal haemostastic response

A

Primary- Platelet Plug formation, platelets, vWF, endothelial wall

Secondary- Fibrin plug formation

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2
Q

What is haemorrhagic diathesis?

A

Unusual susceptibility to bleed

  • Any quantitative or qualitative abnormality inhibition of function
  • Due to platelets, vWF, coagulation factors
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3
Q

Points to ask in bleeding disorders bleeding history?

A
Has patient got a bleeding disorder, 
How severe is it?,
pattern of bleeding, 
Congenital or acquired, 
Mode of inheritance
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4
Q

Key points to ask about with suspected bleeding disorder to ascertain how severe?

A
How appropriate? 
Bruising, 
Epistaxis, 
Post surgical bleeding (how long? Outcome? Dental surgery, tonsillectomy?)
Menorrhagia,
Post-partum haemorrhage, 
Post-trauma,
Spontaneously?
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5
Q

If due to platelets what types of bleeds are you likely to see?

A
Mucosal 
Epistaxis 
Purpura 
Menorrhagia 
GI
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6
Q

If due to coagulation factors what types of bleeds are you likely to see?

A

Articular
Muscle haematoma
CNS (intracranial bleed)

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7
Q

Questions to ask to assess if bleeding disorder is congenital or acquired

A

Previous episodes ?
Age at first visit for bleed ?
Previous surgical challenges ?
Associated history ?

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8
Q

Types of inheritance of haemophilia

A

X-linked

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9
Q

What is haemophilia?

A

Genetic disorder impairing body’s function to make clots

Severity of bleeding depends on the residual coagulation factors activity:
<1% severe
1-5% moderate
5-30% mild

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10
Q

Presentation of haemophilia

A

Haemoarthrosis: Bleeding into joint

Muscle Haematoma: Bleeding into muscle

CNS bleeding: Less commonly seen

Retroperitoneal bleeding

Post-surgical bleeding

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11
Q

Difference between haemophilia A and B

A

A=deficiency in factor 8

B=deficiency in factor 9

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12
Q

Haemophilia clinical complications

A

Synovitis,
Chronic haemophilic arthropathy,
Neurovascular compression (eg compartment syndromes),
Other sequelae of bleeding (eg stroke)

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13
Q

Haemophilia diagnosis

A
Clinical, 
Prolonged APTT,
Normal PT, 
Reduced factor 8 or 9,
Genetic analysis
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14
Q

Haemophilia treatment of bleeding diathesis

A
Coagulation factor replacement, 
DDAVP (causes release of vWF)
Tranexamic acid, 
Prophylaxis,
Gene therapy
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15
Q

Treatment of haemophilia to prevent bleeds

A
Splints, 
Physiotherapy, 
Analgesia, 
Synovectomy, 
Joint replacement
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16
Q

Haemophilia treatment complications

A

Viral infection- HIV, HBV, HCV
Inhibitors- anti FVIII antibodies
DDAVP- MI, hyponatraemia

17
Q

Typical clinical picture of haemophilia

A

Male
6mnts-1yr
Bleeding into joint

18
Q

What is the inheritance type of Von Willebrand Disease

A

Autosomal dominant

19
Q

Presentation of Von Willebrand Disease

A
Mucosal pattern of bleeding:
Easily bruising,
Large bruises,
Frequent/prolonged epistaxis,
Bleeding gums,
Heavy/prolonged bleeding from cuts,
Menorrhagia,
Post partum haemorrhage
20
Q

Von Willebrand Disease types

A

Type I:
Mildest and most common form
Quantitative defect
Don’t make enough

Type II (A,B,M,N):
Qualitative deficiency
Quality of the protein is not good enough
Doesn’t work properly

Type III:
Severe
Complete deficiency

21
Q

Treatment of Von Willebrand Disease?

A

vWF concentrate or DDAVP
Tranexamic acid
Topical applications
OCP

22
Q

Causes of acquired bleeding disorders

A
Thrombocytopenia,
Liver failure, 
Renal failure, 
DIC, 
Drugs- warfarin, heparin, aspirin, clopidogrel, rivaroxaban, apixaban, dabigatran, bivalirudin
23
Q

Causes of thrombocytopenia

A

Decreased production of platelets:

  • marrow failure
  • aplasia
  • infiltration

Increased consumption of platelets:

  • immune ITP
  • non immune DIC
  • hypersplenism
24
Q

Thrombocytopenia clinical presentation

A

Petechia,
Ecchymosis,
mucosal bleeding,
Rare CNS bleeding

25
Q

What does ITP stand for

A

Immune Thrombocytopenic purpura

26
Q

What is Immune thrombocytopenia purpura?

A

Defined as low platelet count with normal bone marrow
And absence of other causes of thrombocytopenia
2 distinct patterns: Acute and Chronic

27
Q

Describe immune ITP

A

Caused by antiplatelet antibodies

Acute:
Children
Following acute infection
Self-limiting purpura

Chronic:
Adults
Mainly women
Fluctuating course

28
Q

What is associated with ITP

A

Infection esp. EBV, HIV
Collagenosis
Lymphoma
Drug induced esp quinine

29
Q

Treatement for ITP

A

Steroids,
IV IgG,
Splenectomy,
Thrombopoietin analogues (Eltrombopag, romiplostim)

30
Q

What coagulation factors are associated with liver

And diagnostic findings of liver failure with regards to bleeding?

A

Factor I, II, VII, IX, X, XI

Prolonged PT and APTT
Reduced fibrinogen

31
Q

Treatment of bleeding in liver failure

A

FFP,

Vitamin K

32
Q

Treatment of haemorrhagic disease of newborn

A

Vitamin K !!