Haemostasis Flashcards

0
Q

What do endothelial cells do to prevent thrombus formation in the absence of injury?

A
Secrete prostacyclin
Secrete nitric oxide
Express heparin sulphate
Express thrombomodulin
Express tissue factor pathway inhibitor
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1
Q

What two processes constitute primary haemostasis?

A

Vasoconstriction

Platelet activation

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2
Q

What does secretion of prostacyclin do?

A

Inhibits platelet activation and aggregation

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3
Q

What does expression of heparin sulphate do?

A

Activates antithrombin

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4
Q

What does thrombomodulin do?

A

Changes thrombin’s affinity away from activation of proclotting factors towards activation of anticoagulant factors

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5
Q

What is the earliest response to vascular injury?

A

Vasospasm

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6
Q

What is the normal lifespan of a platelet?

A

10 days

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7
Q

What does the glycoprotein receptor, GP IIb/IIIb, on platelets bind to?

A

Free fibrinogen and von Willebrand factor

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8
Q

What is the normal activation of platelets dependant on?

A

Von Willebrand factor

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9
Q

What does von Willebrand factor bind to, causing an increase in the substances half-life?

A

Factor VIII (8)

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10
Q

What are the four primary compounds that activate platelets?

A

Collagen
ADP
Thromboxane A2
Thrombin

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11
Q

What are contained in alpha granules in platelets?

A

Fibrinogen
Von Willebrand factor
Factor V (5)

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12
Q

What do platelets release on activation to cause further platelet activation?

A

ADP

Thromboxane A2

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13
Q

What causes shape change in platelets when they are activated?

A

Increase in intracellular calcium

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14
Q

What is factor I?

A

Fibrinogen

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15
Q

What is activated factor II?

A

Thrombin

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16
Q

What is also known as Christmas factor?

A

Factor IX (9)

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17
Q

What is factor III also known as?

A

Tissue factor

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18
Q

What is specific to the extrinsic pathway?

A
Tissue factor
Factor VII (7)
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19
Q

What is specific to the intrinsic pathway?

A
Factor VIII (8)
Factor IX (9)
Factor XI (11)
Factor XII (12)
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20
Q

What is part of the common pathway?

A

Factor X (10)
Thrombin
Fibrin

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21
Q

What are the three main antithrombotic control mechanisms?

A

Antithrombin
Protein C pathway
Tissue factor pathway inhibitor

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22
Q

What does antithrombin inactivate?

A
Thrombin
Factor VIIa
Factor IXa
Factor Xa
Factor XIa
23
Q

What does protein C and it’s cofactor, protein S, inactivate?

A

Factor Va

Factor VIIIa

24
Q

What cleaves fibrin in fibrinolysis?

A

Plasmin

25
Q

What activates conversion of plasminogen to plasmin?

A

Tissue-type plasminogen activator

Urinary-type plasminogen activator

26
Q

What does plasmin cleaving fibrin produce that can be tested?

A

D dimer

27
Q

What are the three inhibitors of fibrinolysis?

A

Plasminogen activator inhibitor 1
Alpha 2 antiplasmin
Thrombin activatable fibrinolysis inhibitor

28
Q

What vitamin is important to the coagulation cascade?

A

Vitamin K

29
Q

What factors is vitamin K involved (needed) in the formation of?

A
Prothrombin
Factor VII
Factor IX
Factor X
Protein C
Protein S
30
Q

What is bleeding from mucous membranes and purpura characteristic of?

A

Bleeding due to thrombocytopenia or abnormal platelet function

31
Q

What three things can cause thrombocytopenia?

A

Reduced platelet production in bone marrow
Excessive peripheral destruction of platelets
Sequestration in enlarged spleen

32
Q

What are the common features of immune thrombocytopenia purpura?

A

Easy bruising
Purpura
Epistaxis
Menorrhagia

33
Q

What are the characteristic features of thrombotic thrombocytopenia purpura?

A
Florid purpura
Fever
Fluctuating cerebral dysfunction
Haemolytic anaemia
Sometimes accompanied with renal failure
34
Q

What commonly precedes ITP in children?

A

Viral infection, eg varicella zoster or measles

35
Q

What is there a lack of in haemophilia A?

A

Factor VIII (8)

36
Q

How is haemophilia A inherited?

A

X-linked

37
Q

What does the severity of bleeding in haemophilia A depend on?

A

Amount of residual coagulation factor activity

38
Q

What are common symptoms of severe haemophilia A?

A

Bleeding into joints - recurrent bleeding can lead to joint deformity and crippling
Bleeding into muscles

39
Q

What are the main laboratory features of haemophilia A?

A
Prolonged APTT
Reduced level of factor VIII
Normal bleeding time
Normal PT
Normal VWF
40
Q

What is haemophilia B also known as?

A

Christmas disease

41
Q

What is haemophilia B a deficiency in?

A

Factor IX (9)

42
Q

What does von Willebrand factor have a role in?

A

Platelet adhesion to damaged subendothelium

Stabilising factor VIII in plasma

43
Q

What is the abnormal in Von Willebrand disease?

A

Deficiency or abnormal von Willebrand factor causing:
Defective platelet function
Factor VIII deficiency

44
Q

What are the typical clinical features of von Willebrand disease?

A

Bleeding following minor trauma or surgery
Epistaxis
Menorrhagia

Haemarthroses are rare

45
Q

What are the main laboratory features of von Willebrand disease?

A
Prolonged bleeding time
Prolonged APTT
Normal prothrombin time
Reduced von Willebrand factor levels
Reduced factor VIII levels
46
Q

What 3 things may cause vitamin K deficiency?

A

Inadequate stores
Malabsorption of vitamin K
Oral anticoagulant drugs

47
Q

What may cause inadequate stores of vitamin K?

A

Haemorrhagic disease of newborn

Severe malnutrition

48
Q

What are the clinical features of vitamin K deficiency?

A

Bruising
Haematuria
Gastrointestinal or cerebral bleeding

49
Q

What are the laboratory features of vitamin K deficiency?

A
Prolonged prothrombin time
Prolonged APTT
Normal bleeding time
Normal factor VIII levels
Normal von Willebrand factor levels
50
Q

What is disseminated intravascular coagulation?

A

Widespread initial thrombosis followed by a tendency to bleed

51
Q

Why does bleeding occur in DIC?

A

Consumption of platelets and coagulation factors

Activation of fibrinolysis

52
Q

Name some causes of DIC.

A
Malignant disease
Septicaemia 
Haemolytic transfusion reactions
Obstetric causes
Trauma, burns, surgery
Other infections eg malaria
Liver disease
Snake bite
53
Q

What is the treatment for DIC?

A

Treat underlying cause
Maintain blood volume and tissue perfusion
Transfuse platelets, plasma, red cell concentrates in patients who are bleeding

54
Q

How does the mutation in Factor V Leiden affect factor V?

A

Factor V is less likely to be cleaved by activated protein C

55
Q

What does Factor V Leiden cause?

A

A tendency to thrombosis, particularly if individual has other thrombosis risk factors, eg oral contraceptive pill, pregnancy

56
Q

What type of genetic conditions are protein S and C deficiency?

A

Autosomal dominant