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 Answer BOF 3.56

 

   

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BOF: 3.56

 

A 30-year-old male presents with a history of feeling tired and passing dark urine. Five days prior to presentation his general practitioner had treated him with co-trimoxazole, as he had been complaining of a sore throat.

Investigations reveal:

Haemoglobin 8.4 g/dL (11.5 – 16.5 g/d)

Serum total bilirubin 107 μmol/L (1 – 22 μmol/L)

The blood film shows multiple bite cells

The most likely cause of this patient’s present problem is:

 

a)      Paroxysmal cold haemoglobinuria

 

b)      Autoimmune haemolytic anaemia

 

c)      Glucose-6-phosphate dehydrogenase deficiency

 

d)      Cold agglutinin disease

 

e)      Paroxysmal nocturnal haemoglobinuria

 

Answer: c)

Bite Cells

Bite cells are red cells from which denatured, precipitated masses of haemoglobin have been pitted by the spleen.

The precipitation is the result of oxidative injury to haemoglobin by drugs or by the denaturation of unstable mutant haemoglobins.

If a large amount of precipitation occurs, the spleen is unable to pit all the precipitate and red cells with precipitate may appear in the peripheral blood.

The precipitate within the red cell is known as a Heinz body.

The patient has a haemolytic anaemia precipitated by treatment with a sulpha containing antibiotic

The most likely underlying cause is glucose-6- phosphate dehydrogenase deficiency

G6PD catalyses reduction of nicotinamide adenine dinucleotide phosphate (NADP) to NADPH

 

NADPH protects cells from oxidative damage

 

Erythrocytes do not generate NADPH in any other way

 

Hence they are more susceptible than other cells to destruction from oxidative stress.

 

Revision Tip

 

Revise G6PD deficiency KEYS to SUCCESS in Medicine page 378-380

 

 

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