Corrections 2

Question 1

What does cryoprecipitate contain?

Answer 1

Factor VIII
Fibrinogen
vWf
Factor XIII

Question 2

What is the major constituent of cryoprecipitate?

Answer 2

Factor VIII

Question 3

What is CML blast transformation?

Answer 3

The final stage of CML

CML transforms until an acute leukaemia, usually AML.

Question 4

Features of blast transformation in CML?

Answer 4

• fever
• fatigue
• SOB
• enlarged spleen
• bone pain
• abdo pain
• night sweats
• weight loss
• bleeding
• infections

Question 5

What is the universal donor of FFP?

Answer 5

AB RhD negative

Question 6

Mechanism of dabigatran?

Answer 6

Direct thrombin inhibitor

Question 7

What investigation should be used to diagnose hereditary spherocytosis?

Answer 7

EMA binding test

Question 8

1st line imaging in suspected multiple myeloma?

Answer 8

Whole body MRI - to assess for bony involvement

Question 9

What is the treatment of choice in TTP?

Answer 9

Plasma exchange

Question 10

Features of TTP?

Answer 10

• fever
• fluctuating neuro signs (microemboli)
• renal failure

Question 11

Blood test results in TTP?

Answer 11

1) Microangiopathic haemolytic anaemia –> schistocytes

2) Thrombocytopenia

Question 12

What are schistocytes on a peripheral blood smear a characteristic feature of?

Answer 12

Microangiopathic hemolytic anemia:

• DIC
• TTP
• HUS
• HELLP
• malfunctioning cardiac valves

Question 13

Causes of TTP?

Answer 13

• post-infection e.g. GI, urinary
• pregnancy
• SLE
• HIV

Question 14

Metabolic abnormalities in tumour lysis syndrome?

Answer 14

• high potassium
• high phosphate
• low calcium

Question 15

What is the platelet threshold for transfusion for patients with severe bleeding, or bleeding at critical sites, such as the CNS?

Answer 15

<100

Question 16

Platelet transfusion thresholds:

1) patients with clinically significant bleeding e.g. haematemesis, melaena, prolonged epistaxis

2) patients with severe bleeding or CNS bleeding

Answer 16

1) <30
2) <100

Question 17

What platelet level should you aim for prior to surgery/invasive procedure?

Answer 17

> 50

Question 18

How should TXA be given in cases of major haemorrhage?

Answer 18

IV bolus followed by slow infusion

Question 19

What is the most common hereditary haemolytic anaemia in people of northern European descent? (e.g. Finland)

Answer 19

Hereditary spherocytosis

Question 20

What is erythema infectiosum caused by?

Answer 20

Parvovirus (‘slapped cheek’)

Question 21

Mx of post-thrombotic syndrome?

Answer 21

Graudated compression stockings

Question 22

Features of post-thrombotic syndrome?

Answer 22

• painful, heavy calves
• pruritus
• swelling
• varicose veins
• venous ulceration

Question 23

What is required to diagnose tumour lysis syndrome?

Answer 23

Laboratory tumour lysis syndrome plus one or more of the following:

1) increase in serum creatinine (1.5x upper limit of normal)

2) cardiac arrhythmia or sudden death

3) seizure

Question 24

A peripherlal blood smear showing spherocytes can be found in what 2 conditions?

Answer 24

1) hereditary spherocytosis

2) autoimmune haemolytic anaemia

Question 25

Pathophysiology of haemophilia?

Answer 25

Interferes with the intrinsic pathway of the coagulation cascade. Factor VIII/IX deficiency --> reduced activation of factor X to Xa --> reduced generation of thrombin --> reduced conversion of fibrinogen (soluble) to insoluble fibrin (provides structural integrity of a clot).

Question 26

Which clotting pathway is affected in haemophilia?

Answer 26

Intrinsic coagulation cascade

Question 27

What clotting study result is one of the hallmarks of haemophilia?

Answer 27

Prolonged APTT (this represents a delay in the intrinsic coagulation pathway).

Question 28

How can clotting studies differentiate between vitamin K deficiency or DIC and haemophillia?

Answer 28

Haemophilia: - prolonged APTT (intrinsic) - normal PT Vitamin K deficiency: - prolonged PT - can have a prolonged APTT

Question 29

What is the most sensitive screening test to detect early or mild vitamin K deficiency?

Answer 29

Prolonged PT

Question 30

Mx of haemophilia?

Answer 30

Replacement of clotting factors: VIII in haemophilia A, IX in haemophilia B

Question 31

What can be used in acute episodes of mild haemophilia A to promote the function of vWf?

Answer 31

Desmopressin

Question 32

Inheritance of thalassaemia?

Answer 32

Autosomal recessive

Question 33

Cause of beta-thalassaemia?

Answer 33

Mutation in globin (HBB) gene on chromosome 11

Question 34

HbA vs HbA2 levels in beta-thalassaemia major?

Answer 34

HbA: absent HbA2: raised (HbF is also raised)

Question 35

What is required for a diagnosis of polycythaemia vera?

Answer 35

1) Mutation in JAK2 and 2) High haematocrit (>0.52 in men, >0.48 in women) OR raised red cell mass (>25% above predicted)

Question 36

What is used to reduce risk of thrombosis in polycythaemia vera?

Answer 36

Aspirin

Question 37

1st line mx of polycythaemia vera?

Answer 37

Venesection

Question 38

What is the mainstay of imaging for staging HL?

Answer 38

PET/CT This combines functional imaging from PET, which detects areas of increased metabolic activity, with anatomical imaging from CT, providing precise localisation of disease sites.

Question 39

What is the initial investigation of choice in suspected polycythaemia vera?

Answer 39

JAK2 mutation screen - this is +ve in approx 95% of PV cases

Question 40

Mechanism of: a) rivaroxaban b) apixaban c) dabigatran d) heparin

Answer 40

a) direct factor Xa inhibitor b) direct factor Xa inhibitor c) direct thrombin inhibitor d) activates antithrombin III

Question 41

What clotting factors does warfarin inhibit?

Answer 41

II, VII, IX and X

Question 42

What is the most likely biochem findings in myeloma (without mets)?

Answer 42

- raised calcium - normal phosphate - normal ALP

Question 43

Is extra-nodal disease more common in HL or non-HL?

Answer 43

Non-HL

Question 44

What medication is often used in the mx of non-HL?

Answer 44

Rituximab

Question 45

What should all patients be screened for prior to receiving treatment with rituximab?

Answer 45

Hep B (can cause reactivation)

Question 46

Why does SLE commonly cause neutropenia?

Answer 46

Autoimmune destruction of neutrophils

Question 47

What is the single most important factor in determining whether cryoprecipitate should be given?

Answer 47

A low fibrinogen level

Question 48

What is cryoprecipitate mainly used in the mx of?

Answer 48

DIC --> indicated for the treatment of hypofibrinogenaemia

Question 49

What blood product may be indicated in: a) prolonged APTT b) prolonged PT c) low fibrinogen

Answer 49

a) FPP b) FPP c) cryoprecipitate