give an example of an acquired vascular abnormality
henoch-schonlein purpura
thrombocytopenia - acquired
causes of peripheral platelet destruction
coagulopathy
- Disseminated Intravascular Coagulation
autoimmune
- Immune thrombocytopenic purpura (ITP)
hypersplenism
platelet functional defects - acquired
drugs (e.g. aspirin, NSAIDs)
renal failure
vWF deficiency - inheritance pattern
autosomal dominant
is vWF generally mild or severe
mild
what is the commonest cause of primary haemostatic failure
thrombocytopenia
causes of thrombocytopenia
multiple factor deficiencies
where are coagulation factors synthesised
hepatocytes
why does liver failure play a part in coagulation factor deficiencies
all coagulation factors are synthesised in hepatocytes
where is vit K absorbed
upper intestine
causes of vit K deficiency
Poor dietary intake
Malabsorption
Obstructive jaundice
Vitamin K antagonists (warfarin)
Haemorrhagic disease of the newborn
causes of DIC
Sepsis
Obstetric emergencies
Malignancy
Hypovolaemic shock
Treatment of DIC
Treat the underlying cause
Replacement therapy
-Platelet transfusions
-Plasma transfusions
-Fibrinogen replacement
haemophilia inheritance pattern
x-linked
what is haemophilia
An X-linked, hereditary disorder in which abnormally prolonged bleeding recurs episodically at one or a few sites on each occasion
what coagulation factor is deficient in haemophilia A
factor VIII
what coagulation factor is deficient in haemophilia B
factor IX
clinical features of severe haemophilia
Recurrent Haemarthroses
Recurrent soft tissue bleeds
-bruising in toddlers
Prolonged bleeding after dental extractions, surgery and invasive procedures