Bleeding Disorders Flashcards
give an example of an acquired vascular abnormality
henoch-schonlein purpura
thrombocytopenia - acquired
- reduced production
- increased destruction
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
- marrow failure
- peripheral destruction
multiple factor deficiencies
- liver failure
- vit K deficiency/warfarin therapy
- complex coagulopathy
-disseminated intravascular coagulation
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