Disorders of Haemostasis Flashcards

1
Q

Give examples of minor bleeding symptoms

A
Easy bruising
Gum bleeding
Frequent nosebleeds (epistaxis)
Bleeding after tooth extraction 
Post-operative
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2
Q

Give examples of minor bleeding symptoms in women

A

Menorrhagia
Post-partum bleeding
Family history

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3
Q

What are the elements of a significant bleeding history

A

Epistaxis not stopped by 10 minutes compression pr requiring medical attention/transfusion
Cutaneous haemorrhage or bruising with no trauma
Prolonged (>15min) bleeding from trivial wounds
Menorrhagia requiring treatment that leads to anaemia
Heavy, prolonged or recurrent bleeding after surgery or dental extraction

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4
Q

Why might abnormal haemostasis occur and give an example of how

A

Lack of a specific factor
Failure of production or increased consumption/clearance

Defective function of a specific factor
Genetic defect or acquire defect (drugs, synthetic defect, inhibition)

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5
Q

Give examples of disorders of primary haemostasis

A
  • thrombocytopenia
  • impaired platelet function
  • vessel wall dysfunction
  • loss or dysfunction of vWF
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6
Q

What may cause thrombocytopenia

A

Bone marrow failure: e.g. leukaemia, vitamin B12 deficiency

Increased use: e.g. disseminated intravascular coagulation (DIC)

Increased clearance: e.g. autoimmune thrombocytopenic purpura

Pooling of platelets in an enlarged spleen

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7
Q

What occurs in autoimmune thrombocytopenic purpura

A

Autoantibodies for platelets, causing their phagocytosis by macrophages

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8
Q

What may cause impaired function of platelets in primary haemostasis

A

Hereditary disorders of glycoproteins: e.g. Bernard-Soulier syndrome, absence of Gp1b

Drugs: e.g. aspirin, NSAIDS, clopidogrel

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9
Q

What can vessel wall dysfunction in primary haemostatic disorders be due to

A
Hereditary connective tissue disorders e.g. hereditary haemorrhagic telangiectasia (Ehlers-Danlos syndrome)
Scurvy
Steroid therapy
Vasculitis
Aging (senile purpura)
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10
Q

What are the functions of VWF in haemostasis

A

Binding to collagen and capturing platelets

Stabilising factor VIII (may be low if VWF is low)

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11
Q

Describe Von Willebrand Disease

A

Hereditary disease of vWF quantity or function
but can be caused by antibodies to vWF
May cause low VIII.

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12
Q

Describe the typical bleeding of primary haemostasis

A
Immediate 
Superficial bleeding into skin and mucous membranes e.g. nosebleeds
Prolonged bleeding from cuts
Epistaxes
Gum bleeding
Menorrhagia 
Easy bruising
Prolonged bleeding after trauma or surgery
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13
Q

What might be the typical bleeding specific to thrombocytopenia and severe VWD

A

thrombocytopenia - petechiae

Severe VWD - haemophilia-like bleeding

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14
Q

Give some hereditary causes of secondary haemostatic disorders and state their effect

A

II (incompatible with life)

XI (more bleeding after trauma)

XII (no - adverse effects)

VIII or IX (haemophilia, see below)

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15
Q

Give some causes of secondary haemostatic disorders

A

Liver disease, because most factors are made there

Dilution of the blood

Anticoagulants: e.g. warfarin

Increased use: e.g. DIC (like for thrombocytopenia)

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16
Q

Describe disseminated intravascular coagulation

A

Generalised activation of coagulation – Tissue factor
Associated with sepsis, major tissue damage, inflammation

Consumes and depletes coagulation factors
Platelets consumed
Activation of fibrinolysis depletes fibrinogen
Deposition of fibrin in vessels causes organ failure

17
Q

Describe the typical bleeding in coagulation disorders (Secondary)

A

Deep bleeding into tissues, muscles, and joints
Delayed bleeding after trauma, which is then prolonged
Often restarts after stopping

18
Q

Which tests can be done for primary and secondary haemostasic disorders

A

Prothrombin time (PT)
Activated partial thromboplastin time (APTT)
Full blood count (platelets)
Tests for specific inhibitors

19
Q

Describe the genetics for haemophilia

A

X-linked recessive

20
Q

Describe the genetics for vWD

A

type 1 and 2 - autosomal dominant

type 3 - autosomal recessive.

21
Q

What are some causes of fibrinolysis disorders

A

hereditary - antiplasmin deficiency

Acquired - drugs (tPA), disseminated intravascular coagulation

22
Q

Describe the genetics of most bleeding disorders

A

Autosomal recessive

23
Q

What is the treatment for abnormal haemostasis from failure of production/function

A

Replace missing factor/platelets
- Prophylactic
- Therapeutic
Stop drugs

24
Q

What is the treatment for abnormal haemostasis from immune destruction

A

immunosuppression

25
Q

What is the treatment for abnormal haemostasis from increased consumption

A

Treat cause

Replace as necessary

26
Q

What can be used in factor replacement therapy

A

Plasma
Cryoprecipitate
Factor concentrates
Recombinant forms of FVIII and FIX

27
Q

Describe plasma and cryoprecipitate for replacement therapy

A

Plasma = contains all coagulation factors

Cryoprecipitate = rich in fibrinogen, FVIII, VWF, FXIII

28
Q

Describe factor concentrates

A

Concentrates available for all factors except factor V.

Prothrombin complex concentrates (PCCs) Factors II, VII, IX, X

29
Q

What are some additional haemostat treatments

A

DDAVP
Tranexamic acid
Fibrin glue/spray

30
Q

Describe desmopressin/DDAVP

A

a vasopressin derivative
Releases endogenous stores of VIII and - vWF, increasing levels 2-5x (VIII more than vWF)
Only useful in mild disorders (due to endogenous store release)

31
Q

Describe transexamic acid

A

Inhibits fibrinolysis
Widely distributed – crosses placenta
Low concentration in breast milk