pass med haem

Question 1

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Answer 1

Factor V Leiden (activated protein C resistance) is the most common inherited thrombophilia, being present in around 5% of the UK population.

It is due to a gain of function mutation in the Factor V Leiden protein. The result of the mis-sense mutation is that activated factor V (a clotting factor) is inactivated 10 times more slowly by activated protein C than normal. This explains the alternative name for factor V Leiden of activated protein C resistance,

Question 2

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Answer 2

Hypersegmented neutrophils on the blood film indicate a megaloblastic anaemia.

Question 3

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Answer 3

Haptoglobin

(Remember haptoglobin binds to free haemoglobin) Decrease
intravascular haemolysis

A decrease in haptoglobin levels can be seen in intravascular haemolysis

Question 4

G6PD deficiency

Answer 4

1. Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the commonest red blood cell enzyme defect.
2. X-linked recessive
3. ↓ G6PD → ↓ glutathione → increased red cell susceptibility to oxidative stress

4.eonatal jaundice is often seen
intravascular haemolysis
gallstones are common
splenomegaly may be present

5. Heinz bodies on blood films. Bite and blister cells may also be seen

Question 5

Hodgkin’s lymphoma

Answer 5

1. presents with a neck mass due to lymphadenopathy, and sometimes weight loss and night sweats, which are termed “B symptoms”.
2. enlarged lymph nodes may become painful on alcohol consumption
3. Reed-Sternberg cell

Question 6

B cell chronic lymphocytic leukaemia

Answer 6

smudge cells (also known as smear cells)

blood film shows that 60% of the white cells are small mature lymphocytes.

Question 7

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Answer 7

Howell-Jolly bodies and siderocytes are typical blood film findings of hyposplenism

Question 8

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Answer 8

Mirror image nuclei = Reed-Sternberg cells