Bleeding Disorders Flashcards
1
Q
What is the normal haemostatic response?
A
- primary
- platelet plug formation
- platelets
- von Willebrand Factor (vWF)
- wall
- secondary
- fibrin plug formation
2
Q
What is haemorrhagic diathesis?
A
- any quantitive or qualitative abnormality
- inhibition of function
- platlets
- vWF
- coagulation factors
3
Q
What are different patterns of bleeding?
A
- platelet type
- mucosal
- epistaxis
- purpura
- menorrhagia
- GI
- coagulation factor
- articular
- muscle haematoma
- CNS
4
Q
What are features of haemophilia A and B?
A
- X-linked
- identicle phenotypes
- 1 in 10,000 + 1 in 60,000
- severity of bleeing depends on residual coagulation factor activity
- < 1% severe
- 1-5% moderate
- 5-30% mild
5
Q
What are signs of haemophilia?
A
- haemarthrosis
- muscle haematoma
- CNS bleeding
- retroperitoneal bleeding
- post surgical bleeding
6
Q
What are complication of haemophilia?
A
- synovitis
- chronic haemophilic arthropathy
- neurovascular compression
- compartment syndomes
- other sequela of bleeding
- stroke
7
Q
What are investigations for haemophilia?
A
- clinical
- prolonged APTT
- normal PT
- dec. FVIII or FIX
- genetic analysis
8
Q
What is the treatment for haemophilia bleeding diathesis?
A
- coagulation factor replacement FVIII/IX
- recombinant products
- DDAVP
- tranexamic acid
- prophylaxis in severe haemophilia
- gene therapy
9
Q
What is the treatment for haemophilia?
A
- splints
- physiotherapy
- analgesia
- synovectomy
- joint replacement
10
Q
What is treated as haemophilia complications?
A
- viral infections
- HIV
- HBV, HCV
- inhibitors
- anti FVIII Ab
- rare in FIX
- DDAVP
- MI
- hyponatraemia (babies)
11
Q
What are features of von Willebrand disease?
A
- common, 1 in 200
- variable severity
- autosomal
- platelet type bleeding (mucosal)
- quantative + qualitative abnormalities of vWF
12
Q
What are the types of von Willebrand disease?
A
- type 1- quantitative deficiency
- type 2 (A, B, M, N)- qualitative deficiency, determined by the site of mutation in relation to vWF function
- type 3- severe (complete) deficiency
13
Q
What is the treatment for von Willebrand disease?
A
- vWF concentrate/DDAVP
- tranexamic acid
- topical applications
- OCP, etc
14
Q
What are causes of aquired bleeding disorders?
A
- thrombocytopenia
- liver failure
- renal failure
- DIC
- drugs
- warfarin, heparin, aspirin, clopidogrel
15
Q
What is thrombocytopenia?
A
- dec. production
- marrow failure
- aplasia
- infiltration
- inc. consumption
- immune ITP
- non-immune DIC
- hypersplenism
16
Q
What are the signs of thrombocytopenia?
A
- petechia
- ecchymosis
- mucosal bleeding
- CNS bleeding (rare)
17
Q
What is immune thrombocytopenic pupura (ITP)?
A
- associated with; infection, collagenosis, lymphoma, drug induced
- blood isolated thrombocytopenia
- marrow
- steroid, IV IgG, splenectomy, thrombopoietin analogues
18
Q
What is haemorrhagic disease of the newborn?
A
- immature coagulation systems
- vitamin K deficient diet (ecp. breast)
- fatal + incapacitating haemorrhage
- completely [reventable by administration of vitamin K at birth
