Bleeding Disorders Flashcards
(27 cards)
What are normal haemostatic mechanisms?
Vessel wall
Platelets
Von Willebrand factor
Coagulation factors
What is the primary haemostatic response?
Platelet plug formation
Need platelets, vWF and vessel wall
What is the secondary haemostatic response?
Fibrin plug formation
When can the haemostatic process go wrong?
Any quantitative or qualitative abnormality
Inhibition of function
Platelets, vWF and coagulation factors
What can be symptoms and signs of history of bleeding?
Bruising, epistaxis, response to challenges (surgeries), menorrhagia, post-partum haemorrhage and post-trauma
What is the bleeding assessment tool used?
ISTH
What is the platelet type bleeding?
Most likely mucosal - epistaxis, purpura, menorrhagia and GI
What is the coagulation factor pattern of bleeding?
Articular bleeding
Muscle haematoma and CNS
Describe haemophilia A and B
Both X linked
Identical phenotypes
A is more common
Severity of bleeding depends on the residual coagulation factor activity (severe is less than 1% and mild is 5-30%)
What are some clinical features of haemophilia?
Hemarthrosis, muscle haematoma, CNS bleeding, retroperitoneal bleeding, and post surgical bleeding
What are some clinical complications of haemophilia?
Synovitis, chronic haemophilic arthropathic, neurovascular compression (compartment syndromes) and other (stroke)
How is haemophilia diagnosed?
Clinical, isolated prolonged APTT, normal PT, reduced FVIII or FIX, and genetic analysis
How is haemophilia treated?
Coagulation factor replacement (factor VIII and IX), DDAVP, tranexamic acid and prophylaxis is important
What are other treatments for haemophilia?
Splints, PT, analgesia, synovectomy and joint replacement
What are some complications of haemophilia treatment?
Viral infections - HIV, HBV and HCV
Inhibitors - anti FVIII Ab
DDAVP - MI and hyponatraemia (babies)
Describe von Willebrand disease
Common 1 in 200
Variable severity, autosomal recessive, platelet type bleeding and quantitative + qualitative abnormalities
What is type 1 Von Willebrand disease?
Quantitative deficiency
What is type 2 Von Willebrand disease?
Qualitative deficiency determined by the site of mutation in relation to vWF function
What is type 3 Von Willebrand disease?
Severe (complete) deficiency
What is the treatment for Von Willebrand disease?
vWF concentrate or DDAVP, tranexamic acid and OCP if menorrhagia
What are some acquired bleeding disorders?
Thrombocytopenia, liver failure, renal failure, DIC, drugs and acquired clotting factor deficiencies
When can thrombocytopenia be seen?
Decreased production - marrow failure, aplasia and infiltration
Increased consumption - immune ITP (platelets), DIC and hypersplenism
What are the classical symptoms of thrombocytopenia?
Petechia, ecchymosis, mucosal bleeding and rare CNS bleeding
Describe immune thrombocytopenia (ITP)
Presents differently in children to adults
Associated with infection - EBV + HIV, lymphoma and drug induced
Isolated thrombocytopenia
