Clotting Disorders Flashcards

1
Q

Give four causes of thrombocytopaenia

A

Marrow failure
Tumour Infiltration
Infection (HIV, EBV)
Drugs (NSAIDs, quinine)

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2
Q

What two infections are associated with thrombocytopaenia?

A

HIV

EBV

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3
Q

Give some drugs that are associated with thrombocytopaenia

A

NSAIDs

Quinine

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4
Q

Presentation of thrombocytopaenia (4)

A

Spontaneous bleeding
Purpura
Menorrhagia
Epistaxis

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5
Q

Investigation findings in thrombocytopaenia

A

Low platelet count

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6
Q

What is the underlying pathophysiology in ITP?

A

Anti-platelet antibodies

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7
Q

What is ITP?

A

Immune Thrombocytopaenic Purpura

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8
Q

Presentation of ITP

  • Kids
  • Adults
A

Kids - sudden, self-limiting purpura: usually following illness or vaccination
Adults - fluctuating course, usually goes into remission then recurs
Bleeding, purpura, menorrhagia

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9
Q

What is the underlying pathophysiology in TTP?

A

Clumping of platelets together, followed by a lack of ADAMSTS13 to breakdown the clot
These thrombosis result in thrombocytopaenia and damage the endothelium

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10
Q

What is TTP?

A

Thrombotic Thrombocytopaenic Purpura

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11
Q

Give four things associated with TTP

A

SLE
Pregnancy
OCP
Infections e.g. HIV

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12
Q

Presentation of TTP (4)

A

Pupura
Fever
AKI
Microangiopathic Haemolytic Anaemia

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13
Q

Investigation findings in TTP (2)

A

Haemolysis on blood film

Raised LDH

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14
Q

Management of TTP

A

Plasma exchange

May need FFP or cyroprecipitate

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15
Q

Investigation findings in ITP

A

Megakaryocytes on blood film

Anti-platelet antibodies

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16
Q

Management of acute ITP

A

Prednisolone

Usually high dose (1mg/kg) to induce remission

17
Q

What is the underlying pathophysiology of vWF deficiency?

A

Low levels of vWF result in reduced ability of platelets to aggregate

18
Q

What is the inheritance of vWF?

What is the exception?

A

Autosomal dominant

Type 3 vWF (complete lack of vWF) is autosomal recessive

19
Q

Presentation of vWF Deficiency

A

Menorrhagia
Easy bruising
Bleeding gums

20
Q

Investigation findings in vWF deficiency

A

Prolonged bleeding time

Prolonged APTT

21
Q

Management of vWF deficiency

A

Tranexamic acid can help with problematic bleeding e.g. menorrhagia

22
Q

What is the pathophysiology of DIC?

A

Inappropriate activation of haemostasis and then failure of haemostasis
Initially thrombi are formed, which consumes clotting factors
This then activates fibrinolysis excessively

23
Q

Presentation of DIC (3)

A

Purpura
Bruising
Bleeding from cannula site

24
Q

Investigation findings in DIC

A

Prolonged APTT
Prolonged PT
Prolonged bleeding time
Thrombocytopaenia

25
Q

Causes of DIC (4)

A

Sepsis
Obstetric emergencies
Malignancy
Shock

26
Q

How are haemophilias inherited?

A

X-linked recessive disorder

27
Q

What is the underlying pathophysiology of haemophilias?

A

Deficiency of clotting factors which results in a bleeding tendency

28
Q

Types of Haemophilia

A

A - lack of factor VIII

B - lack of factor IX

29
Q

What is Type B Haemophilia also called?

What factor is missing?

A

Christmas’ Disease

Factor IX

30
Q

What factor is missing in Type A Haemophilia?

A

Factor VIII

31
Q

Presentation of Haemophilia (3)

A

Prolonged bleeding
Soft tissue bleeds
Haemarthroses

32
Q

Why are joints which have previously bled more likely to re-bleed?

A

Iron irritates the synovium which triggers neovascularisation
These vessels are friable and more likely to bleed

33
Q

What is the underlying pathophysiology of thrombophilia?

A

A lack of the natural anti-coagulants

34
Q

Presentation of thrombophilia

A

Venous thrombosis

35
Q

What are the types of thrombophilia? (4)

A

Factor V Leiden
Protrombin 20210
Protein S Deficiency
Protein C Deficiency

36
Q

What is the mutation in Factor V Leiden Thrombophilia?

A

Missense mutation

37
Q

Management of Thrombophilia

A

Life long warfarin as anti-coagulation

Heparin is used to treat events