Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Pathology > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

how is haemophilia A inherited?

A

X-linked

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

which factor is low in Haemophilia A?

A

Factor VIII

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

clinical features of haemophilia A

A
  • joint and muscle bleeding

- prolonged bleeding after dental extractions

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

what determines the severity of haemophilia A?

A

the extent of the coagulation factor deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

investigations of haemophilia A

A
  • APTT (prolonged)
  • BT and PT (normal)
  • factor VIII (low)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

treatment of haemophilia A

A

factor VIII concentrates

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

which factor is deficient in haemophilia B?

A

factor IX

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

another name for haemophilia B

A

christmas disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

how is von Willebrand disease inherited?

A

autosomal dominant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

what is deficient in von Willebrand disease?

A

von Willebrand factor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

what happens in von Willebrand disease?

A

impaired platelet adherence to the subendothelium

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

clinical features of von Willebrand disease

A
  • post-traumatic bleeding
  • mucosal bleeding
  • haemarthosis (rare)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

what is haemarthosis?

A

bleeding into a joint cavity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

investigations of von Willebrand disease

A
  • bleeding time (prolonged)
  • factor VIII (low)
  • VWF antigen and activity (decreased)
  • platelet aggregation with ristocetin (decreased)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

management of von Willebrand disease

A
  • DDAVP
  • cryoprecipitate
  • intermediate purity factor VIII
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
Q

causes of vitamin K deficiency

A
  • obstructive jaundice
  • warfarin
  • broad spectrum anitbiotics
26
Q

where do you derive vitamin K?

A
  • green vegetables

- bacterial synthesis in the gut

27
Q

why can vitamin K levels be low in newborns?

A
  • liver immaturity
  • lack of gut bacterial synthesis of vitamin
  • low quantities in breast milk
28
Q

investigations of haemorrhagic disease in newborns

A
  • APTT, PT (prolonged)

- fibrinogen (normal)

29
Q

treatment of haemorrhagic disease of newborn

A
  • vitamin K prophylaxis
30
Q

causes of thrombocytopenia

A
  • decreased marrow production

- increased consumption

31
Q

what can cause decreased marrow production in thrombocytopenia?

A
  • marrow infiltration
  • marrow aplasia
  • post radio/chemotherapy
32
Q

what can cause increased consumption in thrombocytopenia?

A
  • transfusion
  • sepsis
  • massive haemorrhage DIC
  • hypersplenism
  • microangiopathic haemolytic anaemias (TTP, HUS)
  • autoimmune (ITP, HIT)
33
Q

what happens in idiopathic thrombocytopenic purpura?

A

autoantibodies directed against GBIIIbIIa receptors

34
Q

causes of idiopathic thrombocytopenic purpura?

A
  • idiopathic
  • viral infections
  • drugs/vaccines
  • lymphomas
  • CLL
35
Q

clinical features of childhood-type of ITP

A
  • acute presentation
  • viral aetiology
  • outcome self-limiting
36
Q

clinical features of adult-type of ITP

A
  • chronic presentation
  • usually idiopathic
  • lifelong condition
37
Q

investigations of ITP

A
  • marrow aspirate (normal)

- blood film (no abnormal cells)

38
Q

treatment of ITP

A
  • immunosuppression

- immunomodulation

39
Q

management of ITP

A
  • steroids
  • steroid-sparing agents (thiopurine)
  • splenectomy
  • rituximab
  • lymphoma type chemotherapy
  • avoid platelet transfusions if possible
  • IVIG
  • anti-D

Pathology (21 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions