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Haemostasis Flashcards

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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?

Study These Flashcards
A 
Thrombin
Factor VIIa
Factor IXa
Factor Xa
Factor XIa
23
Q

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

Study These Flashcards
A

Factor Va

Factor VIIIa

24
What cleaves fibrin in fibrinolysis?
Plasmin
25
What activates conversion of plasminogen to plasmin?
Tissue-type plasminogen activator | Urinary-type plasminogen activator
26
What does plasmin cleaving fibrin produce that can be tested?
D dimer
27
What are the three inhibitors of fibrinolysis?
Plasminogen activator inhibitor 1 Alpha 2 antiplasmin Thrombin activatable fibrinolysis inhibitor
28
What vitamin is important to the coagulation cascade?
Vitamin K
29
What factors is vitamin K involved (needed) in the formation of?
``` Prothrombin Factor VII Factor IX Factor X Protein C Protein S ```
30
What is bleeding from mucous membranes and purpura characteristic of?
Bleeding due to thrombocytopenia or abnormal platelet function
31
What three things can cause thrombocytopenia?
Reduced platelet production in bone marrow Excessive peripheral destruction of platelets Sequestration in enlarged spleen
32
What are the common features of immune thrombocytopenia purpura?
Easy bruising Purpura Epistaxis Menorrhagia
33
What are the characteristic features of thrombotic thrombocytopenia purpura?
``` Florid purpura Fever Fluctuating cerebral dysfunction Haemolytic anaemia Sometimes accompanied with renal failure ```
34
What commonly precedes ITP in children?
Viral infection, eg varicella zoster or measles
35
What is there a lack of in haemophilia A?
Factor VIII (8)
36
How is haemophilia A inherited?
X-linked
37
What does the severity of bleeding in haemophilia A depend on?
Amount of residual coagulation factor activity
38
What are common symptoms of severe haemophilia A?
Bleeding into joints - recurrent bleeding can lead to joint deformity and crippling Bleeding into muscles
39
What are the main laboratory features of haemophilia A?
``` Prolonged APTT Reduced level of factor VIII Normal bleeding time Normal PT Normal VWF ```
40
What is haemophilia B also known as?
Christmas disease
41
What is haemophilia B a deficiency in?
Factor IX (9)
42
What does von Willebrand factor have a role in?
Platelet adhesion to damaged subendothelium | Stabilising factor VIII in plasma
43
What is the abnormal in Von Willebrand disease?
Deficiency or abnormal von Willebrand factor causing: Defective platelet function Factor VIII deficiency
44
What are the typical clinical features of von Willebrand disease?
Bleeding following minor trauma or surgery Epistaxis Menorrhagia Haemarthroses are rare
45
What are the main laboratory features of von Willebrand disease?
``` Prolonged bleeding time Prolonged APTT Normal prothrombin time Reduced von Willebrand factor levels Reduced factor VIII levels ```
46
What 3 things may cause vitamin K deficiency?
Inadequate stores Malabsorption of vitamin K Oral anticoagulant drugs
47
What may cause inadequate stores of vitamin K?
Haemorrhagic disease of newborn | Severe malnutrition
48
What are the clinical features of vitamin K deficiency?
Bruising Haematuria Gastrointestinal or cerebral bleeding
49
What are the laboratory features of vitamin K deficiency?
``` Prolonged prothrombin time Prolonged APTT Normal bleeding time Normal factor VIII levels Normal von Willebrand factor levels ```
50
What is disseminated intravascular coagulation?
Widespread initial thrombosis followed by a tendency to bleed
51
Why does bleeding occur in DIC?
Consumption of platelets and coagulation factors | Activation of fibrinolysis
52
Name some causes of DIC.
``` Malignant disease Septicaemia Haemolytic transfusion reactions Obstetric causes Trauma, burns, surgery Other infections eg malaria Liver disease Snake bite ```
53
What is the treatment for DIC?
Treat underlying cause Maintain blood volume and tissue perfusion Transfuse platelets, plasma, red cell concentrates in patients who are bleeding
54
How does the mutation in Factor V Leiden affect factor V?
Factor V is less likely to be cleaved by activated protein C
55
What does Factor V Leiden cause?
A tendency to thrombosis, particularly if individual has other thrombosis risk factors, eg oral contraceptive pill, pregnancy
56
What type of genetic conditions are protein S and C deficiency?
Autosomal dominant

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