Bleeding Disorders Flashcards

1
Q

how is haemophilia A inherited?

A

X-linked

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2
Q

which factor is low in Haemophilia A?

A

Factor VIII

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3
Q

clinical features of haemophilia A

A
  • joint and muscle bleeding

- prolonged bleeding after dental extractions

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4
Q

what determines the severity of haemophilia A?

A

the extent of the coagulation factor deficiency

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5
Q

investigations of haemophilia A

A
  • APTT (prolonged)
  • BT and PT (normal)
  • factor VIII (low)
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6
Q

treatment of haemophilia A

A

factor VIII concentrates

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7
Q

which factor is deficient in haemophilia B?

A

factor IX

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8
Q

another name for haemophilia B

A

christmas disease

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9
Q

how is von Willebrand disease inherited?

A

autosomal dominant

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10
Q

what is deficient in von Willebrand disease?

A

von Willebrand factor

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11
Q

what happens in von Willebrand disease?

A

impaired platelet adherence to the subendothelium

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12
Q

clinical features of von Willebrand disease

A
  • post-traumatic bleeding
  • mucosal bleeding
  • haemarthosis (rare)
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13
Q

what is haemarthosis?

A

bleeding into a joint cavity

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14
Q

investigations of von Willebrand disease

A
  • bleeding time (prolonged)
  • factor VIII (low)
  • VWF antigen and activity (decreased)
  • platelet aggregation with ristocetin (decreased)
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15
Q

management of von Willebrand disease

A
  • DDAVP
  • cryoprecipitate
  • intermediate purity factor VIII
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16
Q

what is DDAVP?

A

desmopression (stops bleeding in von Willebrand disease and Haemophilia A)

17
Q

characteristics of factor XII deficiency

A
  • prolonged clotting time

- no clinically significant bleeding

18
Q

characteristics of factor XI deficiency

A
  • mild bleeding tendency

- prolonged bleeding episodes after surgery, dental procedures or trauma

19
Q

characteristics of factor XIII deficiency

A
  • severe bleeding tendency

- normal coagulation tests

20
Q

what happens to coagulation in scenarios of liver disease?

A
  • thrombocytopenia
  • folate deficiency
  • alcoholic toxic effect on haemolysis
  • deficiency of vitamin K clotting factors
  • decreased clearance of activated clotting factors
  • decreased fibrinogen synthesis and dysfibrinoginaemia
  • portal hypertension causing varices
  • more sensitive to triggers of DIC
21
Q

features of renal disease coagulopathy

A
  • qualitative platelet defect
  • prolonged bleeding time due to decreased platelet adhesion and aggregation
  • anaemia
  • deficiency of half molecular weight multimers despite increased VWF
  • mild thrombocytopenia due to decreased platelet survival
22
Q

management of renal-associated coagulopathy

A
  • correction of anaemia
  • DDAVP
  • dialysis (may improve bleeding time)
23
Q

what happens to coagulation in renal disesase?

A
  • heparin effect; may develop ATIII deficiency
  • vitamin K deficiency due to malnutrition
  • loss of low molecular weight clotting factors through glomeruli
  • tendency to chronic compensated DIC due to fibrin formation in glomeruli
24
Q

what happens when a patient gets massive transfusions?

A
  • blood deficient in clotting factors and platelets
  • replacement of blood volume in 24hrs causes bleeding tendency
  • dilution of clotting factors and dilutional thrombocytopenia
25
causes of vitamin K deficiency
- obstructive jaundice - warfarin - broad spectrum anitbiotics
26
where do you derive vitamin K?
- green vegetables | - bacterial synthesis in the gut
27
why can vitamin K levels be low in newborns?
- liver immaturity - lack of gut bacterial synthesis of vitamin - low quantities in breast milk
28
investigations of haemorrhagic disease in newborns
- APTT, PT (prolonged) | - fibrinogen (normal)
29
treatment of haemorrhagic disease of newborn
- vitamin K prophylaxis
30
causes of thrombocytopenia
- decreased marrow production | - increased consumption
31
what can cause decreased marrow production in thrombocytopenia?
- marrow infiltration - marrow aplasia - post radio/chemotherapy
32
what can cause increased consumption in thrombocytopenia?
- transfusion - sepsis - massive haemorrhage DIC - hypersplenism - microangiopathic haemolytic anaemias (TTP, HUS) - autoimmune (ITP, HIT)
33
what happens in idiopathic thrombocytopenic purpura?
autoantibodies directed against GBIIIbIIa receptors
34
causes of idiopathic thrombocytopenic purpura?
- idiopathic - viral infections - drugs/vaccines - lymphomas - CLL
35
clinical features of childhood-type of ITP
- acute presentation - viral aetiology - outcome self-limiting
36
clinical features of adult-type of ITP
- chronic presentation - usually idiopathic - lifelong condition
37
investigations of ITP
- marrow aspirate (normal) | - blood film (no abnormal cells)
38
treatment of ITP
- immunosuppression | - immunomodulation
39
management of ITP
- steroids - steroid-sparing agents (thiopurine) - splenectomy - rituximab - lymphoma type chemotherapy - avoid platelet transfusions if possible - IVIG - anti-D