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MCD Haematology > Abnormalities of haemostasis > Flashcards

Abnormalities of haemostasis Flashcards

1
Q

What are the two main types of abnormal haemostasis?

A

Lack of a specific factor

Defective function of a specific factor

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2
Q

What can cause you to lack a specific factor?

A

Failure of production- congenital or acquired

Increased consumption/clearance

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3
Q

What can cause a defective function of a specific factor?

A 
Genetic defect
Acquired defect (more common) e.g. drugs, synthetic defect and inhibition
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4
Q

What is thrombocytopenia?

A

Low number of platelets

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5
Q

What causes thrombocytopenia?

A

Failure of platelet production by megakaryocytes- Bone marrow failure e.g. leukaemia and B12 deficiency
Shortened half life of platelets- Accelerated clearance e.g. autoimmune thrombocytopenia, disseminated intravascular coagulation
Increased pooling of platelets in enlarged spleen

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6
Q

What else could disorders of primary haemostasis be caused by except from thrombocytopenia?

A

Impaired function of platelets- absence of storage granules or glycoproteins

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7
Q

What can cause impaired function of platelets?

A

Acquired due to drugs e.g. aspirin, NSAIDs, clopidogrel

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8
Q

What hereditary platelet defects are there?

A

Glanzann’s thrombasthenia
Bernard Soulier syndrome
Storage pool disease

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9
Q

What causes Glanzann’s thrombasthenia?

A

Absent glycoprotein 2b/3a

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10
Q

What does the absence of glycoprotein 2b/3a cause?

A

Lack of platelet aggregation

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11
Q

What causes Bernard Soulier syndrome?

A

Lack of glycoprotein 1b

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12
Q

What does the lack of glycoprotein 1b cause?

A

Platelets can’t bind to von Willebrand factor

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13
Q

What is storage pool disease?

A

Problem with storage granules so they can’t be released adequately

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14
Q

What is Von Willebrand disease?

A

Hereditary decrease in quantity and/or function

It can be acquired due to an antibody

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15
Q

What are the two functions of vWF in haemostasis?

A

Binding to collagen and capturing platelets

Stabilising factor 8

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16
Q

What are the three types of VWD?

A

Type 1- Deficiency of VWF but functions normally
Type 2- VWF is made but doesn’t function normally
Type 3- VWF isn’t made at all

17
Q

What are some examples of inherited (rare) disorders of primary haemostasis that affect the vessel wall?

A

Hereditary haemorrhage telangiectasia
Ehlers-Dalos syndrome
Other connective tissue disorders

18
Q

What are some examples of acquired disorders of primary haemostasis that affect the vessel wall?

A 
Scurvy
Steroid therapy
Atrophy of supporting tissues of blood vessels
Ageing (senile purpura)
Vasculitis
19
Q

What is typical primary haemophilia bleeding like?

A 
Immediate
Prolonged bleeding from cuts
Epistaxes
Gum bleeding
Menorrhagia
Easy bruising
Prolonged bleeding after trauma
Thrombocytopenia gives rise to petechiae
20
Q

Why can you have haemophilia type bleeding in severe VWD?

A

VWF levels are very low so factor 8 will also be very low

21
Q

What is haemophilia?

A

Failure to generate fibrin to stabilise platelet plug- Lack of factor 8 or 9 resulting in impaired thrombin generation

22
Q

What is the genomics of haemophilia like?

A

It is an X linked condition

23
Q

How severe is deficiency of prothrombin (factor 2) compared to haemophilia?

A

Deficiency of prothrombin is lethal but haemophilia is severe but compatible with life

24
Q

How severe are deficiency of factor 11 and factor 12

A

Factor 11- Bleed after trauma but not spontaneously

Factor 12- No excess bleeding

25
Q

How can deficiency of coagulation factors be acquired?

A

Liver disease
Dilution
Anti-coagulant drugs e.g. warfarin

26
Q

What are two examples of increased consumption leading to disorders of coagulation?

A

Disseminated intravascular coagulation

Autoimmune antibodies

27
Q

What is disseminated intravascular coagulation?

A

Generalised activation of coagulation- tissue factor is exposed and causes activation of cascade

28
Q

What is DIC associated with?

A

Sepsis, major tissue damage and inflammation

29
Q

What does DIC do?

A

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

30
Q

What is typical bleeding in coagulation disorders?

A

Superficial cuts don’t bleed
Bruising is common
Nosebleeds are rare
Spontaneous bleeding is deep into muscles and joints
Bleeding after trauma is delayed and prolonged

31
Q

In what condition should intramuscular injections be avoided and why?

A

Haemophilia because it can cause deep bleeding patterns

32
Q

What tests are there for coagulation disorders?

A

Screening tests
Prothrombin time- extrinsic path disorders
Activated partial thromboplastin time- intrinsic path disorders
Platelet count
Factor assays
Tests for inhibitors

33
Q

What bleeding disorders are not detected by routine clotting tests?

A 
Mild factor deficiencies
Von Willebrand disease
Factor 8 deficiency
Platelet disorders
Excessive fibrinolysis 
Vessel wall disorders
Metabolic disorders
34
Q

What hereditary and acquired disorders of fibrinolysis are there?

A

Rare
Hereditary- Antiplasmin deficiency
Acquired- Drugs such as tap and disseminated intravascular coagulation

35
Q

How is abnormal homeostasis where production failure is responsible treated?

A

Replace missing factor/platelets
Prophylactic
Therapeutic
Stop drugs

36
Q

How is abnormal haemostasis where immune destruction is responsible treated?

A

Immunosuppression

Splenectomy for autoimmune thrombocytopenia

37
Q

How is abnormal haemostasis where increased consumption is responsible treated?

A

Treat cause

Replace as necessary

38
Q

If you’re give someone factor replacement therapy, what are the different sorts and what do they contain?

A

Plasma- contains all coagulation factors
Cryoprecipitate- Fibrinogen, factor 8, vWF and factor 13
Factor concentrates- available for all factors except 5

39
Q

How is desmopressin (DDAVP) used to treat haemostasis?

A

Vasopressin analogue which makes the endothelial cells release their own VWF that is stored- good for mild VWD

MCD Haematology (11 decks)

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