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Year 3 Immunology > 12. Bleeding Disorders > Flashcards

12. Bleeding Disorders Flashcards

1
Q

What forms the primary heamostasis

A

Platelet plug formation, platelets, vWF, wall

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2
Q

What forms the secondary heamostasic response

A

Fibrin

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3
Q

What is haemorrhaged diathesis?

A

Any quantitative or qualitative abnormality. Inhibition of function.

Can involve platelets, vWF, coagulation factors

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4
Q

What important points are important to investigate when taking a bleeding history

A 
Has the patient actually got a bleeding disorder?
How severe is the disorder
Pattern of bleeding
Congenital disorder? Acquired disorder?
Mode of inheritance
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5
Q

How do you take a bleeding history?

A 
Bruising
Epistaxis
Post surgical bleeding
Mennorrhagia
Post partum heamorrhage
Post trauma

(Dental surgery, circumcision, tonsillectomy, appendectomy)

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6
Q

What classifies a severe bleeding disorder?

A

Spontaneous bleeding that occurs every 3/4 weeks. Can be into the body

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7
Q

What is platelet/mucosal bleeding

A

Caused by platelet factors or vFW deficiencies presents with

Epistaxis, purpurem mennorrhagia, GI

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8
Q

What is coagulation factor bleeding?

A

Bleeds into muscles, joints and articulate spaces

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9
Q

What are the features of mucosal bleeding?

A

Petichae, big bruises called e-something?

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10
Q

What is the classical presentation of people with coagulation disorders

A

Knee/ankle swelling. Hot, tender, swollen.

Coagulation factor 8 deficiency most common, called type 1 disorder.

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11
Q

How can you differentiate between congenital and acquires bleeding disorders?

A

Previous surgeries, did you bleed?

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12
Q

What is autosomal dominant inheritance?

A

Affected family members of each sex in each generation

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13
Q

What is hemophilia A?

A

Most common type of haemophilia, X linked, deficiency of coagulation factor 8. Severity of bleeding depends on the residual coagulation factor activity

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14
Q

What is heamophilia B

A

Christmas disease, arises from defect from coagulation factor 9. Again severity of bleeding depends on residual coagulation factor

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15
Q

What is the worst joint to be damaged by haemophilia?

A

Ankle

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16
Q

What are the clinical features of haemophilia?

A 
Haemathrosis- blood in joint, tingles, throbs and becomes sore, unbearable pain
Muscle heamatoma
CNS bleeding
Retroperitoneal bleeding
Post surgical bleeding
17
Q

What are the complications of haemophilia?

A

Synovitis
Chronic haemophillicnarthropathy
Neurovascular compression
Other sequelae of bleeding (stroke)

18
Q

How do you diagnose haemophilia?

A

Clinical, prolonged APTT, normal PT reduced FVIII or FIX

Genetic analysis

19
Q

How do you treat heamophilia?

A

Coagulation factor replacement
Entirely recombinant products
DDAVP- releases stored vFW and FVIII from endothelium
Tranexamic Acid- proheamostatic agent
Emphasis on prophalxysis
Gene therapy
Mezuzimab- monoclonal antibody- does the job of FVIII

20
Q

What are the contraindication of DDAVP

A

Young children- hyponatraemia

People with cardiac complications

21
Q

What is Von willebrands disease?

A

Autosomal dominant disease, typically mucosal bleeding.

22
Q

How do you treat type I vWF

A

Tranexamic acid and DDAVP
vFW concentrate
Contraception- girls with menahhoregia

23
Q

What is thrombocytopenia?

A

Decreased blood cells

This can be chased by marrow failure, aplasia and infiltration

Increased consumption causes immune ITP, non immune DIC and hypersplenism

24
Q

What is the clinical pattern of thrombocytopenia

A

Petechia
EEhymosis
Mucosal bleeding
Rare CNS bleeding

25
Q

Where’s the best place to look for petichae?

A

The ankles

26
Q

What is ITP?

A

Seen in children post infection e.g. EBV

Can also been seen in lymphoma, drug induced or connective tissue disorders

27
Q

How do you treat ITP?

A

Steroids, IV IgG, splenectomy

Thrombopoitein analogues

28
Q

How does liver failure cause abnormalities in the blood?

A

Coagulation factors produced in liver as well as natural anticoagulants.

This leads to reduced fibrinogen and increase PT
Vit K dependant factors are also reduced

29
Q

What is haemorrhaged disease of the newborn?

A

Every baby that is born has an immature coagulation system?
Vit K deficient diet causes heamorrhagic disease of the newborn
They get massive heamorrhags

Year 3 Immunology (26 decks)

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