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Haematology and immunology > Bleeding disorders > Flashcards

Bleeding disorders Flashcards

1
Q

Do patients with problems in primary haemostasis present in the same way as those with problems in those with secondary haemostasis?

A

No

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2
Q

What is haemorrhagic diathesis?

A

Any quantitative or qualitative abnormality

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3
Q

Inhibition of what can be associated with abnormal bleeding?

A

Platelets
vWF
Coagulation factors

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4
Q

How is severity of disorder determined?

A

Depends what it takes to make you bleed

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5
Q

What is important to find out about bleeding history?

A 
Has the patient actually got a disorder
Severity
Pattern of bleeding
Congenital or acquired
Mode of inheritance
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6
Q

What are features of bleeding?

A 
Bruising
Epistaxis
Post-surgical bleeding
Menorrhagia
Post-partum haemorrhage
Post-trauma
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7
Q

What is the most important question to ask?

A

Previous surgery - dental as well, circumcision

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8
Q

What may be an indication of mild bleeding disorder?

A

After minor procedures ie immunisation

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9
Q

What is an indication of severe bleeding disorder?

A

Spontaneous bleeding

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10
Q

What is platelet type bleeding?

A

Dysfunction in platelets and vWF

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11
Q

How may platelet type bleeding present?

A

Epistaxis
Purpura
Menorrhagia
GI

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12
Q

How may coagulation factor type bleeding present?

A

Articular
Muscle haematoma
CNS

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13
Q

How can you determine if bleeding disorder is congenital or acquired?

A

Previous episodes?
Age at first event
Previous surgical challenges
Associated history

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14
Q

What should be asked about family history?

A

Family members with similar history

Sex - determine sex linked disorder

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15
Q

Are haemophilia A and B X linked?

A

Yes

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16
Q

How is severity of haemophilia A and B determined?

A

Coagulation factor activity:
5-30% = mild
1-5% = moderate
<1% = severe

17
Q

Does moderate haemophilia A and B bleed spontaneously?

A

No

18
Q

What are clinical features of haemophilia?

A 
Haemarthrosis
Muscle haematoma
CNS bleeding
Retroperitoneal bleeding
Post surgical bleeding
19
Q

What joint is worst effected in haemophilia?

A

Ankle joint

20
Q

What drives syonvial hyertrophy in haemophilia?

A

Blood cells being phagocytosed excites inflamamtory cells to produce cytokines

21
Q

What are clinical complications of haemophilia?

A

Synovitis
Chronic haemohilic arthropathy
Neurovascular compression - compartment syndromes
Other sequelae of bleeding

22
Q

How is haemophilia diagnosed?

A

Clinical diagnosis
Prolonged APTT, normal PT, reduced factor VIII or factor IX
Genetic analysis

23
Q

How is haemophilia treated?

A 
Coagulation factor replacement
Now almost entirely recombinant products
DDAVP
Tranexamic acid
Emphasis on prophylaxis in severe haemophilia
Gene therapy?
24
Q

What are complications of haemophilia treatment?

A

Viral infection - HIV, HBV, HCV, etc
Anti factor VIII antibodies produced, rarely factor IX
DDAVP - MI, hyponatraemia in babies

25
Q

What type of bleeding is vWF disease?

A

Platelet type

26
Q

What affects qualitative disorder?

A

Where the mutation arises on the gene

27
Q

How can vWF be treated?

A

vWF concentrate or DDAPV
Tranexamic acid
Topical applications
OCP for menorrhagia

28
Q

What can cause decreased production in thrombocytopenia?

A

Marrow failure
Aplasia
Infiltration

29
Q

What can cause increased consumption in thrombocytopenia?

A

Immune ITP
Non immune DIC
Hypersplenism

30
Q

What is ITP associated with?

A

Infections
Collagenosis
Lymphoma
Drug induced

31
Q

How is ITP treated?

A

Steroids
IV IgG
Splenectomy
Thrombopoietin analogues

32
Q

What is affected in liver failure?

A

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

Prolonged prothrombin time, reduced fibrinogen

33
Q

What causes haemorrhagic disease of the newborn?

A

Baby has immature coagulation systems

Particularly vit K deficiency

34
Q

How is haemorrhagic disease of the newborn prevented?

A

Vit K injection immediately on birth

Haematology and immunology (19 decks)

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