Bleeding disorders

Question 1

Give types + examples of vascular abnormalities?

Answer 1

Hereditary deficiencies
(e.g. Marfans, ehlers dahnlos)

Acquired (vasculitis e.g.
Henoch-Schonlein purpura)

Question 2

Give the 2 types of thrombocytopenia?

Answer 2

Hereditary
Acquired

Question 3

What is thrombocytopenia?

Answer 3

A low platelet count

Question 4

What are 2 possible causes of acquired thrombocytopenia?

Answer 4

Reduced platelet production - marrow failure

Increased peripheral platelet destruction

Question 5

What 2 things care known to cause acquired platelet functional defects?

Answer 5

Drugs
(aspirin, clopidogrel, NSAIDs)

Renal failure

Question 6

What are 3 well known causes of peripheral platelet destruction?

Answer 6

Coagulopathy (e.g. disseminated intravascular coagulation)

Autoimmune (e.g. immune thrombocytopenic purpura)

Hypersplenism (uraemia)

Question 7

What is Von Willebrand factor (vWF) deficiency?

Answer 7

A common hereditary autosomal dominant condition in which there is not enough Von Willebrand factor being produced

Question 8

What is the most common cause of primary haemostatic failure?

Answer 8

Thrombocytopenia

Question 9

How do multiple clotting factor deficiencies typically occur?

Give a well known example?

Answer 9

Generally acquired

Disseminated intravascular coagulation

Question 10

How does a single clotting factor deficiency typically occur?

Give a well known example?

Answer 10

Generally hereditary

Haemophilia

Question 11

What 3 causes are there for multiple factor deficiencies?

Answer 11

Liver failure

Vitamin K deficiency / warfarin therapy

Complex coagulopathy (disseminated intravascular coagulation)

Question 12

What carboxylates coagulation factors II, VII, IX and X ?

Answer 12

Vitamin K

Question 13

What is the main source of vitamin K?

Answer 13

Diet - leafy greens (kale)

Question 14

What does vitamin K require for its absorption?

Answer 14

Bile salts

Question 15

Give 5 causes of vitamin K deficiency?

Answer 15

Poor dietary intake
Malabsorption
Obstructive jaundice
Vitamin K antagonists (warfarin)
Haemorrhagic disease of newborn

Question 16

What is disseminated intravascular coagulation?

Answer 16

The excessive and inappropriate activation of the haemostatic system

Question 17

What occurs as a result of disseminated intravascular coagulation?

Answer 17

Microvascular thrombus formation which can cause end organ failure

Clotting factor consumption also leads to bruising, purpura and generalised bleeding

Question 18

Low levels of coagulation factor VII causes a prolongation of what?

Answer 18

Prothrombin time (PT)

Question 19

Low levels of coagulation factor IXa causes a prolongation of what?

Answer 19

Activated partial thromboplastin time (aPTT)

Question 20

What converts fibrin to FDPs?

Answer 20

Plasmin

Question 21

What are FDPs?

Answer 21

Fibrin degradation products

Question 22

Give 4 causes of disseminated intravascular coagulation?

Answer 22

Sepsis
Obstetric emergencies
Malignancy
Hypovolaemic shock

Question 23

How should disseminated intravascular coagulation be treated?

Answer 23

Treating the underlying cause

Replacement therapy
e.g. platelet / plasma transfusions, fibrinogen replacement

Question 24

What is haemophilia?

Answer 24

An X-linked hereditary disorder in which abnormally prolonged bleeding recurs episodically at one or a few sites on each occasion

Question 25

What are 2 common types of haemophilia?

Answer 25

Haemophilia A Haemophilia B

Question 26

Who are more likely to be affected by haemophilia?

Answer 26

Men ( it is an X-linked condition)

Question 27

What is the most common type of haemophilia?

Answer 27

Haemophilia A

Question 28

What is haemophilia A?

Answer 28

A factor VIII deficiency

Question 29

What is haemophilia B?

Answer 29

A factor IX deficiency

Question 30

Which vessels are affected in haemophilia?

Answer 30

Medium to large blood vessels

Question 31

What test suggests haemophilia clinically?

Answer 31

A raised activated partial thromboplastin time (aPTT)

Question 32

Give 2 clinical features of severe haemophilia?

Answer 32

Recurrent hemarthroses (bleeding into a joint) Recurrent soft tissue bleeds

Question 33

Give a clinical feature of moderate haemophilia?

Answer 33

Prolonged bleeding after dental extractions, surgery and invasive procedures

Question 34

What suggests multiple factor deficiency in a patient?

Answer 34

An acquired pattern of disorder Multiple prolonged screening tests

Question 35

What suggests a single factor deficiency in a patient?

Answer 35

An isolated prolonged aPTT

Question 36

How does mild thrombocytopenia present?

Answer 36

Easy bruising Prolonged bleeding time Nosebleeds Bleeding gums Heavy periods Haematuria Rectal bleeding

Question 37

What are the two most concerning spontaneous bleeds that can occur in thrombocytopenia?

Answer 37

Intracranial haemorrhage Gastrointestinal bleeding

Question 38

What is immune thrombocytopenic purpura?

Answer 38

A condition in which antibodies are created against platelets causing an immune response against them leading to thrombocytopenia

Question 39

What is purpura?

Answer 39

Non-blanching regions caused by bleeding under the skin Looks like 'pin pricks'

Question 40

How is immune thrombocytopenic purpura managed?

Answer 40

Prednisolone IV immunoglobulins Rituximab Splenectomy

Question 41

What is thrombotic thrombocytopenic purpura?

Answer 41

A condition in which small thrombi develop in the small vessels by using up platelets

Question 42

What does thrombotic thrombocytopenic purpura cause?

Answer 42

Thrombocytopenia Purpura Tissue ischaemia End-organ damage

Question 43

How is thrombotic thrombocytopenic purpura managed?

Answer 43

Plasma exchange Steroids Rituximab

Question 44

How will patients with Von Willebrand disease present?

Answer 44

Unusually easy and prolonged bleeding heavy bleeding Epistaxis Heavy periods - menorrhagia

Question 45

How is Von Willebrand's disease diagnosed?

Answer 45

Family history Clinical history Bleeding assessment

Question 46

How can Von Willebrand's disease be managed?

Answer 46

Desmopressin Tranexamic acid Von Willebrand factor infusion Factor VIII and Von Willebrand factor infusion

Question 47

What should be given to patients with heavy menstrual periods if Von Willebrand's is likely?

Answer 47

Tranexamic acid

Question 48

What is haemarthrosis?

Answer 48

Spontaneous bleeding into joints

Question 49

How is haemophilia diagnosed?

Answer 49

Bleeding scores Coagulation factor assays Genetic testing

Question 50

How can haemophilia be treated?

Answer 50

Intravenous infusions of the impacted clotting factors