Haematology

Question 1

A 21 year old woman with transfusion dependent beta thalassaemia normally has a transfusion every two weeks.

She has recently moved into your care and no previous blood results are available.

Pre-Transfusion bloods show a Hb of 76 (low) and a WCC of 23 (Normal Range: <11)

She is clinically well, apyrexial. She denies losing weight or night sweats. Her appetite is good.

What blood test should be ordered to further investigate the raised white cell count given her history?

Answer 1

Reticulocyte count

Question 2

Hyperkalaemia ECG changes

Answer 2

p wave flattening, hyperacute T waves and a broadened QRS

Question 3

What blood test is a measure of fibrin degredation products and may reflect activity of the body’s anticoagulant system?

Answer 3

D Dimer

Question 4

What is the term given to a group of genetic condition which affect the synthesis of the haem molecule, which is vital for the function of haemoglobin, peroxidase and P450 enzymes?

Answer 4

Porphyria

Question 5

What is the reversal agent for rivaroxaban?

Answer 5

Andexanet alfa

Question 6

A medical student is unsure about what cell she is looking at under the microscope. She is looking a peripheral blood film.

She describes a cell that looks plentiful on the film and has a multisegmented nucleus. It is approximately twice the size of the red cells she can see on the film.

What cell is she describing?

Answer 6

Neutrophil

Question 7

What staging system is used to stage Hodgkin’s Lymphoma?

Answer 7

Lugano staging classification

Question 8

What inherited condition, after cystic fibrosis, is the most common cause of pancreatic exocrine dysfunction in children?

Answer 8

Shwachman Diamond syndrome

Question 9

What prothrombotic condition is associated with thrombocytopenia and immune complex formation?

Answer 9

Heparin induced thromboycytopenia

Question 10

What is the classical finding on a full blood count for a patient suspected of dyskeratosis congenita?

Answer 10

pancytopenia

Question 11

Heparin induced thrombocytopenia is associated with the formation of antibodies directed against what autoantigen?

Answer 11

Platelet factor 4

Question 12

What is the most common reaction to transfusion of packed red blood cells?

Answer 12

Fever

Question 13

A 70 year old man is seen in clinic. He describes a history of progressive enlargement of a “lump” in his groin.

USS reveals it to be a lymph node and a biopsy is taken.

What is the likely histopathological type of lymphoma that will be seen on biopsy?

Answer 13

Diffuse Large B Cell Lymphoma

Question 14

What are inclusions of denatured haemoglobin within erythrocytes commonly called when seen in a peripheral blood film?

Answer 14

Heinz bodies

Question 15

A 18 year old Greek man has just returned from a holiday to Malawi and arranges a GP appointment.

He said during the holiday he turned yellow, his urine turned brown and he felt more tired than normal.

He has no relevant past medical history, is up to date on her vaccinations and took antimalarial prophylaxis as directed.

The doctor suspects an intravascular haemolysis. Blood tests reveal a normocytic anaemia with unconjugated bilirubinemia. Levels of G6PD were normal. DAT negative.

What is the most likely reason for the intravascular haemolysis?

Answer 15

Glucose-6-phosphate dehydrogenase deficiency- absolute levels of G6PD may be normal but G6PD in RBCs may be LOW!!

Question 16

A patient with metastatic ovarian cancer is investigated for unilateral lower limb swelling and is confirmed to have a DVT.

What is the most appropriate form of anticoagulation?

Answer 16

DOAC

Question 17

Which chemical ion is required to convert Factor IX to Factor X in the intrinsic pathway of the clotting cascade and Factor VII to Factor X in the extrinsic pathway of the clotting disorder?

Answer 17

Calcium

Question 18

What protein is typically defective in hereditary elliptocytosis?

Answer 18

Spectrin

Question 19

A 14 year old boy is seen by a haematologist.

He has been referred due to concerns from his GP about a clotting disorder, as he has had recent unexplained petechiae, nosebleeds, tiredness and fatigue.

Bloods from the GP showed a pancytopenia.

The haematologist performs a full physical examination and notes the presence of longitudinal ridging in the nails and areas of whitish grey discolouration in the mouth. There are fine, end inspiratory crackles at both lung bases.

The haematologist suspects a primary bone marrow disorder.

What is the most likely diagnosis?

Answer 19

Dyskeratosis Congenita

Question 20

What scoring system can be used to calculate the probability of developing heparin induced thrombocytopenia?

Answer 20

4T scoring system

Question 21

What molecule binds free haemoglobin in the bloodstream and may be used in the investigation of haemolysis?

Answer 21

Haptoglobin

Question 22

A patient with an eGFR of 10 is confirmed to have a deep vein thrombosis in the ambulatory emergency care department.

Two weeks later, he is asked by his GP what blood thinning medication he is taking. He is not sure but recalls it is a small white tablet.

What anticoagulant is he likely to be taking?

Answer 22

If there is profound renal failure, a non renally cleared oral anticoagulant would be preferred - generally warfarin. This requires INR monitoring.

Question 23

What score is used to aid clinical decision making with respect to DVT risk?

Answer 23

Wells score

Question 24

What is the reversal agent for dabigatran?

Answer 24

Idarucizumab

Question 25

Which cofactor is required for synthesis of the anticoagulant proteins C and S?

Answer 25

Vitamin K