Clotting Disorders

Question 1

Give four causes of thrombocytopaenia

Answer 1

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

Question 2

What two infections are associated with thrombocytopaenia?

Answer 2

HIV

EBV

Question 3

Give some drugs that are associated with thrombocytopaenia

Answer 3

NSAIDs

Quinine

Question 4

Presentation of thrombocytopaenia (4)

Answer 4

Spontaneous bleeding
Purpura
Menorrhagia
Epistaxis

Question 5

Investigation findings in thrombocytopaenia

Answer 5

Low platelet count

Question 6

What is the underlying pathophysiology in ITP?

Answer 6

Anti-platelet antibodies

Question 7

What is ITP?

Answer 7

Immune Thrombocytopaenic Purpura

Question 8

Presentation of ITP

• Kids
• Adults

Answer 8

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

Question 9

What is the underlying pathophysiology in TTP?

Answer 9

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

Question 10

What is TTP?

Answer 10

Thrombotic Thrombocytopaenic Purpura

Question 11

Give four things associated with TTP

Answer 11

SLE
Pregnancy
OCP
Infections e.g. HIV

Question 12

Presentation of TTP (4)

Answer 12

Pupura
Fever
AKI
Microangiopathic Haemolytic Anaemia

Question 13

Investigation findings in TTP (2)

Answer 13

Haemolysis on blood film

Raised LDH

Question 14

Management of TTP

Answer 14

Plasma exchange

May need FFP or cyroprecipitate

Question 15

Investigation findings in ITP

Answer 15

Megakaryocytes on blood film

Anti-platelet antibodies

Question 16

Management of acute ITP

Answer 16

Prednisolone

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

Question 17

What is the underlying pathophysiology of vWF deficiency?

Answer 17

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

Question 18

What is the inheritance of vWF?

What is the exception?

Answer 18

Autosomal dominant

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

Question 19

Presentation of vWF Deficiency

Answer 19

Menorrhagia
Easy bruising
Bleeding gums

Question 20

Investigation findings in vWF deficiency

Answer 20

Prolonged bleeding time

Prolonged APTT

Question 21

Management of vWF deficiency

Answer 21

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

Question 22

What is the pathophysiology of DIC?

Answer 22

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

Question 23

Presentation of DIC (3)

Answer 23

Purpura
Bruising
Bleeding from cannula site

Question 24

Investigation findings in DIC

Answer 24

Prolonged APTT
Prolonged PT
Prolonged bleeding time
Thrombocytopaenia

Question 25

Causes of DIC (4)

Answer 25

Sepsis Obstetric emergencies Malignancy Shock

Question 26

How are haemophilias inherited?

Answer 26

X-linked recessive disorder

Question 27

What is the underlying pathophysiology of haemophilias?

Answer 27

Deficiency of clotting factors which results in a bleeding tendency

Question 28

Types of Haemophilia

Answer 28

A - lack of factor VIII | B - lack of factor IX

Question 29

What is Type B Haemophilia also called? | What factor is missing?

Answer 29

Christmas' Disease | Factor IX

Question 30

What factor is missing in Type A Haemophilia?

Answer 30

Factor VIII

Question 31

Presentation of Haemophilia (3)

Answer 31

Prolonged bleeding Soft tissue bleeds Haemarthroses

Question 32

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

Answer 32

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

Question 33

What is the underlying pathophysiology of thrombophilia?

Answer 33

A lack of the natural anti-coagulants

Question 34

Presentation of thrombophilia

Answer 34

Venous thrombosis

Question 35

What are the types of thrombophilia? (4)

Answer 35

Factor V Leiden Protrombin 20210 Protein S Deficiency Protein C Deficiency

Question 36

What is the mutation in Factor V Leiden Thrombophilia?

Answer 36

Missense mutation

Question 37

Management of Thrombophilia

Answer 37

Life long warfarin as anti-coagulation | Heparin is used to treat events