haemostasis - inherited coagluative disorders Flashcards
normal homesostasis steps
1) localised vasoconstriction at site of injury
Happen in parallel:
Primary
2) adhesion of platelets to damaged vessel wall and formation of a platelet aggregate or plug
Secondary
3) activation of coagulation cascade leading to fibrin formation, reinforcing the platelet plug
Then
4) activation of the fibrinolytic system which digests the haemostatic plug, re establishing vascular patency
platlets
small non nucleated cells
fragmetns of megakaryocyte cytoplasm
essential for primayr haemostatic plug
thrombocytopenia
reduced platlet count
thrombocytotsis
increased platlet count
coaglulation cascade is initiated by
tissue factor damage
factor 7a
once coagulation is initiated what is triggered
- 10 to 10 a
- 9 to 9a, factor 8 co factor to amplify 10 to 10a
- form factor 2a (thrombin)
- this catalyses fibrinogen (soluble) to fibrin(insoluble)
types of bleeding
primary haemostasis
secondary haemostasis
primary haemostasis
- involves vessel wall, platelets and von Willebrand factor
- present with purpura, bruising, bleeding from cuts, heavy periods and surgical bleeding
what does Von willebrand factor do
sticks platelets to vessel wall)
secondary haemostasis
- involves coagulation cascade
- presents with joint and muscle haematomas and surgical bleeding
- bruising is not prominent
menorrhagia
heavy menstraul periods
epistaxis
nosebleed
haemoptysis
coughing blood
haematemesis
vomiting blood
haematuria
blood in urine