Try it
Biochemistry · Purine catabolism: tumour lysis syndrome
Sample itemA 10-year-old boy with Burkitt lymphoma has high uric acid and is about to receive chemotherapy. Rasburicase is planned. Which test must be done first, and why?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
B. G6PD activity, because hydrogen peroxide from urate oxidase action can cause haemolysis
Humans lack urate oxidase, so uric acid is the end product of purine catabolism. Rasburicase is recombinant urate oxidase that converts uric acid to the far more soluble allantoin, producing hydrogen peroxide as a by-product. G6PD-deficient red cells lack NADPH to detoxify peroxide and may haemolyse or form methaemoglobin, so rasburicase is contraindicated.
Option by option
A. TPMT activity, because rasburicase is a thiopurine
TPMT testing is for thiopurines such as azathioprine and 6-mercaptopurine.
B. G6PD activity, because hydrogen peroxide from urate oxidase action can cause haemolysis (correct)
Correct. Hydrogen peroxide generation makes G6PD deficiency a contraindication.
C. DPD activity, because rasburicase is a fluoropyrimidine
DPD testing is for fluoropyrimidines such as fluorouracil and capecitabine.
D. HGPRT activity, because rasburicase is a purine salvage drug
Rasburicase is an enzyme that degrades urate, and does not depend on HGPRT.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
More Biochemistry questions
- Nucleotide metabolismA 4-year-old boy has intellectual disability, dystonia, self-mutilation and hyperuricaem…
- VitaminsA patient with megaloblastic anaemia and mild neurological signs has raised serum methyl…
- Amino acid metabolismA baby has a mousy odour, fair skin and developmental delay. Which enzyme is deficient?
- Glycogen storage diseasesA child has severe fasting hypoglycaemia, hepatomegaly, lactic acidosis and hyperuricaem…