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?

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
What type of bleeding is vWF disease?
Platelet type
26
What affects qualitative disorder?
Where the mutation arises on the gene
27
How can vWF be treated?
vWF concentrate or DDAPV Tranexamic acid Topical applications OCP for menorrhagia
28
What can cause decreased production in thrombocytopenia?
Marrow failure Aplasia Infiltration
29
What can cause increased consumption in thrombocytopenia?
Immune ITP Non immune DIC Hypersplenism
30
What is ITP associated with?
Infections Collagenosis Lymphoma Drug induced
31
How is ITP treated?
Steroids IV IgG Splenectomy Thrombopoietin analogues
32
What is affected in liver failure?
Factor I, II, V, VII, VIII, IX, X, XI | Prolonged prothrombin time, reduced fibrinogen
33
What causes haemorrhagic disease of the newborn?
Baby has immature coagulation systems | Particularly vit K deficiency
34
How is haemorrhagic disease of the newborn prevented?
Vit K injection immediately on birth