Formative

Question 1

Does “ uncontrolled production of essentially normally functioning blood cells” describe secondary polycythaemia or polycythaemia rubra vera

Answer 1

Polycythaemia rubra vera

- in secondary this is controlled by high epo driven by the secondary cause eg hypoxia

Question 2

What is a Clonal B cell disorder usually resulting in a large number of circulating malignant cells?

Answer 2

chronic lymphocytic leukaemia: excess of small mature lymphocytes in the blood

• acute leukaemia = block of differentiation
• chronic leukaemia = failure of cell death

Question 3

Mechanism of sickle cell disease

Answer 3

point mutation in the beta chain (of globin gene) and this predisposes the haemoglobin to polymerisation resulting in sickled cells and reduced red cell survival.

Question 4

Mechanism of myelodysplasia

Answer 4

Acquired DNA mutations in haematopoietic stem cells

Question 5

Which drug

a) is an ADP agonist?
b) irreversibly inactivates cyclooxygenase 1?
c) is a highly selective direct inhibitor of activated factor X?

Answer 5

a) clopidogrel
b) aspirin
c) rivaroxaban

Question 6

Haemoglobin analysis identifies the presence of raised Hb A2.

Answer 6

beta thalassaemia trait

Question 7

Family history of haemolytic disorder, normocytic anaemia Blood film shows polychromasia and red cells with loss of central pallor.

Answer 7

hereditary spherocytosis

Question 8

Causes of hereditary haemophilia

Answer 8

Factor V Leiden, antithrombin deficiency, protein C deficiency, protein S deficiency

Question 9

What protein does rituximab target?

Answer 9

Rituximab is a humanised monoclonal antibody directed against CD20, expressed on B cells and B cell lymphomas.

Question 10

What protein does imatinib target?

Answer 10

Imatinib is a tyrosine kinase inhibitor that is inhibits BCR-ABL-1 protein unique to chronic myeloid leukaemia.

Question 11

48 year old man becoming breathlessness two hours after an infusion of fresh frozen plasma.

Answer 11

transfusion related acute lung injury: caused by anti- leucocyte antibodies present in the donation that bind to the patients white cells and cause acute lung injury by degranulation of the affected neutrophils in the lungs. Pulmonary infiltrates are seen on CXR.

Question 12

Investigate

a) haemoglobinopathy
b) raised haemoglobin w/o history of secondary polycythaemia
c) acute leukaemia

Answer 12

a) Haemoglobin analysis by high performance liquid chromatography (HPLC)
b) JAK2 gene mutation analysis
c) immunophenotyping

Question 13

Blood film showing macroovalocytes and hypersegmented neutrophils is indicative of what

Answer 13

b12/folate deficiency

Question 14

pancytopenia + b12/folate deficiency

Answer 14

pernicious anaemia

Question 15

The blood film shows an excess of platelets with some giant forms.

Answer 15

essential thrombocythaemia

Question 16

What is vitamin K responsible for?

Answer 16

bile salt absorption and carboxylation of clotting factors

Question 17

Is vitamin K water-soluble?

Answer 17

no

Question 18

In which condition would you see prolonged prothrombin time, prolonged partial thromboplastin time, low platelet count, low fibrinogen.

Answer 18

DIC

Question 19

Treat autoimmune haemolytic anaemia

Answer 19

steroid (1mg/kg of prednisolone per day) and folic acid. Steroids suppress the autoimmune process and folic acid supplements prevent deficiency which can occur when the red cell turnover is high (evidenced by the reticulocytosis).

Question 20

A 21 year old man has 4 months of a gradually enlarging neck lump. His appetite is poor and he has lost 5 kg in weight recently. He has been experiencing night sweats.He has a single, non-tender, 3 cm mass in the left anterior triangle of the neck.

Which investigation is most likely to give a definitive diagnosis? 
A. Bone marrow aspirate
B. CT scan of chest, abdomen and pelvis 
C. Epstein–Barr virus serology 
D. Excision biopsy 
E. Peripheral blood smear

Answer 20

D. excision biopsy

this is hodgkins lymphoma

Question 21

A 65 year old woman has increasing lethargy and a sore, red tongue. She takes levothyroxine sodium 75 micrograms once daily but no other medication.She has vitiligo and looks pale. Her GP suspects that she has anaemia and requests a blood count and blood film.

Which is the most likely abnormality on the blood film? 
A. Blast cells 
B. Macrocytosis 
C. Pancytopenia 
D. Reticulocytosis
 E. Spherocytosis

Answer 21

B. macrocytosis

–> this woman has B12 deficiency as indicated by lethargy, anaemia, glossitis possiby due to pernicious anaemia which would have autoimmune association..

Question 22

A child with parvovirus develop jaundice and anaemia, what is the diagnosis?

Answer 22

hereditary spherocytosis