Bleeding Disorders and Coagulopathies Flashcards

1
Q

Where does heparin work?

A

Multiple sites, including intrinsic system

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2
Q

How is warfarin measured?

A

INR

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3
Q

How is heparin measured?

A

Platelet count

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4
Q

What does APTT measure?

A

Heparin
Factor VIII
Factor IX

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5
Q

What constitutes a coagulation profile?

A

Platelet count
APTT
PT

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6
Q

What tests can measure dabigatran levels?

A

Thrombin clotting time (TCT)

Drug levels

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7
Q

What tests can measure rivaroxaban and apixaban levels?

A

Anti-Xa levels

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8
Q

What is a common treatment if a person is too anticoagulated?

A

Prothrombinex

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9
Q

What is the lifespan of platelets?

A

8-9 days

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10
Q

How can the effects of clopidogrel and aspirin be reversed?

A

Platelet transfusion

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11
Q

If platelet transfusion fails, what else can be done to reverse the effects of clopidogrel and aspirin?

A

Desmopressin

Recombinant factor VIIa

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12
Q

What is the clinical evaluation of bleeding disorders?

A
Age at onset
Spontaneous/post-traumatic
Sites/frequency of bleeds/quantitation of bleeding
Bleeding stressors; eg:
- Tooth extractions
- Surgery
FHx
Medications
Therapy received and response
Convincing evidence of "excessive bleeding"
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13
Q

What does von Willebrand factor do?

A

Mediates platelet adhesion at site of injury

Stabilises factor VIII in circulation

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14
Q

What is type 1 von Willebrand disease?

A

Partial vWF deficiency

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15
Q

What is type 2 von Willebrand disease?

A

2A - decreased affinity of vWF for platelets due to decrease in multimers
2B - increased affinity of vWF for platelets due to vWF mutation > thrombocytopaenia
2M - decreased platelet binding due to vWF mutation
2N - defective factor VIII binding site > decreased factor VIII levels

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16
Q

What is type 3 von Willebrand disease?

A

Complete vWF deficiency

17
Q

When is von Willebrand disease treated?

A

Only if symptomatic, rarely prophylactically

18
Q

What is the treatment of von Willebrand disease?

A
Desmopressin
- Useful for minor procedures
- Effective in type 1
- Causes hyponatraemia
Replacement therapy
19
Q

What is the symptomatic treatment of von Willebrand disease?

A

Antifibrinolytics - inhibit fibrinolysis > prolong thrombus formation time
- TXA
Oral contraceptives/Mirena to manage periods
Fibrin glue - for nosebleeds

20
Q

What are the investigations for von Willebrand disease?

A
May be mildly elevated APTT
Initial tests
- Factor VIII levels
- vWF Ag levels
- vWF activity/function test
- Blood group - group O have lower levels
More specialised tests to subtype
21
Q

What are the investigations for haemophilia?

A
Hb
May be mildly elevated APTT
Normal INR
Initial tests
- Factor VIII and IX levels
- Likely to be levels of 5-30%
Genetic studies
22
Q

What are the clinical manifestations of haemophilia A?

A
Haemarthrosis
Subcutaneous and IM haematomas
Psoas and retroperitoneal haematomas
Traumatic bleeding
- Delayed bleeding, especially in tooth extractions
- Slow wound healing
23
Q

What is the course and prognosis of haemophilia A?

A

Nearly normal lifespan with factor VIII replacement

24
Q

What are the differential diagnoses for haemophilia A?

A

Haemophilia B

von Willebrand’s disease, especially type 2N

25
Q

What is the treatment for haemophilia A?

A

Factor VIII replacement
- Prophylactically in children with severe form
Desmopressin, if mild

26
Q

What is the course and prognosis of haemophilia B?

A

Nearly normal lifespan with factor IX replacement

27
Q

Which of the two is more prevalent: haemophilia A or B?

A

A

28
Q

What is the treatment for haemophilia B?

A

Prothrombinex
Recombinant factor IX therapy
- Given prophylactically in children with severe form

29
Q

What are the investigation results in immune thrombocytopaenia purpura?

A

Thrombocytopaenia

Abs to several surface platelet Ags

30
Q

What is the treatment for immune thrombocytopaenia purpura?

A
Initially high dose steroids
Avoid platelet transfusions
IV Ig if bleeding
Further treatments
- Splenectomy
31
Q

What sort of bleeding occurs in thrombocytopaenia?

A
Skin and mucous membranes
- Petechiae
- Ecchymosis
- Haemorrhagic vesicles
- Gingival bleeding and epistaxis
Menorrhagia
GI bleeding
Intracranial bleeding
32
Q

What is the investigation conducted when APTT is prolonged?

A

Mixing study = equal parts normal pooled plasma added to patient plasma
Significant correction > factor deficiency
No correction > inhibitor present

33
Q

What further tests are indicated if there is correction of APTT in a mixing study?

A

Specific factor assays

  • VIII
  • IX
  • XI
  • XII
34
Q

What further tests are indicated if there is no correction of APTT in a mixing study?

A

Lupus anticoagulant

35
Q

What effect does lupus anticoagulant have on clotting?

A

In vivo - increases clotting

In vitro - increases bleeding

36
Q

What effect does quinine have on clotting?

A

Can induce lupus inhibitors

37
Q

What happens in consumptive coagulopathy?

A

Severe bleeding > all coagulants used up > bleeding

38
Q

What will normal screening tests miss?

A

Mild von Willebrand’s disease
Mild haemophilia
Factor XIII deficiency
Platelet function disorders