Haemostasis and Thrombosis Flashcards
The patient has a bleeding tendency if there is a problem with the following structures/cells/factors:
Endothelial surface
Platelets
Von Willebrand Factor
Coagulation factors
The patient has a clotting tendency if reduced or absent:
Natural anticoagulants
What do platelets release during the first response to injury: blood vessel constriction?
Serotonin (5HT)
Thromboxane A2
When the vessel wall is damaged, what is exposed?
Collagen
How can platelets bind to the exposed collagen of the endothelium?
Directly by GP1a (glycoprotein) receptor
Through von Willebrand factor using GP1b receptor
Which clotting factor is von Willebrand factor a main carrier of?
VIII
How does von Willebrand factor help platelets bind together to form a platelet plug?
vWF binding to GP1b platelet receptor leads to the release of fibrinogen which works together with vWF to add more platelets
What is the purpose of the common pathway in the coagulation cascade?
Formation of fibrin mesh
What is involved in the common pathway cascade?
CF10a activates CF5
CF5a activates thrombin and then breaks down fibrinogen into fibrin
What test is the intrinsic pathway of the coagulation cascade represented by?
aPTT blood test
What is involved in the intrinsic pathway cascade that goes on to activate the common pathway?
CF12a activates CF11 followed by CF9 then CF8
Where does the tissue factor that activates coagulation in the body come from?
Activated endothelium
White cells in the bloodstream during infection or sepsis
What happens during the extrinsic pathway of the coagulation cascade which then leads to the common pathway?
Tissue factor binds to and activates CF7
What do we need for healthy coagulation?
Platelets - normal number & normal function
Von Willebrand Factor
Coagulation factors
Clot stability
What steps to take when meeting a patient with a known bleeding disorder?
- Patient should tell you
- Registered at the local haemophilia centre
- Carry haemorrhagic states card/necklace/bracelet
- Bleeding history should be consistent with clinical severity of disorder
What is vWF for?
Help platelets bind to endothelial surface
Help platelets bind together to form a platelet plug
What are coagulation factors needed for?
Form fibrin mesh
What is haemophilia?
Genetically inherited X-linked condition
Congenital deficiency in factor 8 (Haem. A) and factor 9 (Haem. B)
Unable to stabilise the platelet plug as fibrin strands cannot be formed properly
Why is the vast majority of women with mutation for haemophilia less likely to be affected with symptoms/problems?
Usually, they have the mutation on only one X chromosome and the other one will be unmutated
How do boys get affected by haemophilia?
They inherit their mother’s mutated X chromosome
Y chromosome unable to counter the mutation
Which haemophilia is more common?
Haemophilia A
What is the concentration of coagulation factor (8 or 9) that will determine the diagnosis of haemophilia?
<50%
What is the concentration of the coagulation factor for a patient with severe haemophilia presenting spontaneous bleeding episodes predominantly in the joints of muscles?
<1%
What is the range of the coagulation factor concentration for patients presenting with severe bleeding episodes after surgery and slight bleeding episodes after minor trauma?
5 to 25%
What is the range of the coagulation factor concentration for patients presenting with severe bleeding episodes even after slight trauma?
1 to 5%
What will young patients with haemophilia that don’t get treated develop?
Severe arthritis and arthropathy in their joints