Haematology

Question 1

Apart from diabetes mellitus, what else may cause an elevated HbA1c?

Answer 1

Thalassaemia - the beta chain of Hb is glycated, but in alpha-thalassaemia, there is an excess of beta chains, thus falsely elevating HbA1c
(shortened RBC lifespan or increased turnover = decreased HbA1c)

Question 2

What is thalassaemia?

Answer 2

Autosomal recessive disorder
Genetic mutation causing malformation of either alpha or beta chains of haemoglobin
More common in Asian and African people
May provide resistance to malaria

Question 3

Differentials for splenomegaly.

Answer 3

Removal of Defective RBCs: thalassaemia
Immune Hyperplasia: EBV, CMV, toxoplasmosis, HIV, malaria, RA, SLE, haemolytic anaemia
Portal Hypertension: cirrhosis
Extramedullary Haematopoiesis, Infiltrations: leukaemia, myeloproliferative disease

Question 4

1 unit of whole blood will raise a patient’s haemoglobin by how much?

Answer 4

10g/L

Question 5

How is multiple myeloma diagnosed?

Answer 5

Serum protein electrophoresis for paraprotein/M protein/monoclonal immunoglobulin

Question 6

When can a patient with O- blood be given O+ red cells?

Answer 6

When there is no history of anti-D
Postmenopausal females
Males

Question 7

What is deficient in Haemophilia A, B and C?

Answer 7

A - Factor VIII
B - Factor IX
C - Factor XI

Question 8

How is APTT and PT affected in haemophilia and VWD?

Answer 8

In both, PT will be normal.
In haemophilia, APTT will be prolonged.
In VWD, APTT will only be prolonged if there is reduced Factor VIII.

Question 9

Briefly describe the aetogenesis of multiple myeloma.

Answer 9

1. Premalignant MGUS
2. Smouldering myeloma
3. Multiple myeloma

Question 10

How is multiple myeloma diagnosed?

Answer 10

Serum protein electrophoresis: M protein
Bone marrow biopsy: >10% plasma cells
Evidence of end-organ damage

Question 11

What is the classical presentation of haemophilia?

Answer 11

Haemarthrosis
Bleeding with minima trauma
Multiple ecchymoses

Question 12

What is the peak age of ALL?

Answer 12

Early childhood

Question 13

What is the most common acute leukaemia in adults?

Answer 13

AML

Question 14

Which leukaemia is associated with Auer rods?

Answer 14

AML

Question 15

Which leukaemias are associated with the Philadelphia chromosome?

Answer 15

CLL

ALL (poor prognosis)

Question 16

In what disease do you find Reed-Sternberg cells?

Answer 16

Hodgkin’s lymphoma

Question 17

Which leukaemia is associated with smudge cells?

Answer 17

CLL

Question 18

Which leukaemia is associated with Auer rods?

Answer 18

AML

Question 19

What is the most common leukaemia occurring in children?

Answer 19

ALL - 80%

AML - 20%

Question 20

What are the final few steps in the coagulation cascade?

Answer 20

Extrinsic or intrinsic pathways convert X to Xa
Factor Xa converts prothrombin (II) to thrombin (IIa)
Thrombin converts fibrinogen (I) to fibrin (Ia)
Fibrin binds platelets to form a clot

Question 21

Which clotting factors are also known as fibrinogen and fibrin?

Answer 21

I and Ia

Question 22

Which clotting factors are also known as prothrombin and thrombin?

Answer 22

II and IIa

Question 23

Which type of haemophilia is common in Ashkenazi jews?

Answer 23

Haemophilia C

Question 24

Which clotting factor is deficient in haemophilia A?

Answer 24

Factor VIII

Question 25

Which clotting factor is deficient in haemophilia B (Christmas disease)?

Answer 25

Factor IX

Question 26

Which clotting factor is deficient in haemophilia C?

Answer 26

Factor XI

Question 27

What is the most common hereditary coagulopathy?

Answer 27

Von Willebrand’s Disease

Question 28

What investigations should be ordered for a patient with suspected haemophilia?

Answer 28

CBE (normal platelets)
APTT (prolonged)
INR/PT (normal)
Factor VIII and IX Assay
vWF Activity Level and Antigen (if factor VIII deficient)

Question 29

What investigations should be ordered for a patient with suspected von Willebrand’s disease?

Answer 29

CBE (normal platelets)
APTT (prolonged if reduced factor VIII)
INR/PT (normal)
vWF Antigen (positive)
vWF Activity (reduced)
Factor VIII assay (may be reduced)
Specialised vWF Assays (to determine type of vWD)

Question 30

What is the classic triad of Felty’s Syndrome?

Answer 30

Rheumatoid Arthritis
Neutropenia
Splenomegaly