Paeds Cases · haematology-oncology-and-transfusion
Tumour lysis syndrome and oncologic emergencies: Case
Clinical long case of a seven-year-old boy with a Burkitt lymphoma and a high tumour lysis risk, covering the risk assessment, the prophylaxis with the hyperhydration and the rasburicase before the first dose, the recognition and the management of the tumour lysis syndrome by the Cairo-Bishop definition, the airway-first principle for the anterior mediastinal mass, and the urgent imaging and steroid for the spinal cord compression.
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Framing the case
This seven-year-old boy with the Burkitt lymphoma sits at the very top of the tumour lysis risk. The large abdominal mass, the lactate dehydrogenase of three thousand, and the elevated urate together mark a high tumour burden and a high cell turnover, and the start of the chemotherapy will release a torrent of the intracellular contents. The framework that organises the case is the recognition that the tumour lysis is predictable and preventable, and the first decision is to put the prophylaxis in place before the first dose of the chemotherapy. [3]
The risk assessment and the prophylaxis
The boy is high risk by the Cairo stratification, because the Burkitt lymphoma has the very highest cell turnover of any childhood tumour, with a doubling time of around twenty-four hours. The prophylaxis is the foundation of the management, and it begins before the first dose. The hyperhydration with an isotonic fluid that contains no potassium is run at two to three litres per square metre per day to maintain a urine output above two millilitres per kilogram per hour. The fluid is potassium-free because the child is at risk of the hyperkalaemia. [1]
The rasburicase, a recombinant urate oxidase that converts the existing uric acid into the soluble allantoin, is given at zero point one five to zero point two milligrams per kilogram, and the glucose-six-phosphate-dehydrogenase status is checked first, because the rasburicase is contraindicated in the deficiency and causes a severe haemolysis and a methaemoglobinaemia. The biochemistry of the potassium, the phosphate, the calcium, the creatinine, and the urate is monitored every four to six hours for the first twenty-four to forty-eight hours, and the renal replacement therapy is kept on standby. [1][9]
References7ShowHide
- [1]Howard SC, Avagyan A, Workeneh B, Pui CH Tumour lysis syndrome Nat Rev Dis Primers, 2024.PMID 39174582
- [2]Cairo MS, Bishop M Tumour lysis syndrome: new therapeutic strategies and classification Br J Haematol, 2004.PMID 15384972
- [3]Cairo MS, Coiffier B, Reiter A, et al. Recommendations for the evaluation of risk and prophylaxis of tumour lysis syndrome (TLS) in adults and children with malignant diseases Br J Haematol, 2010.PMID 20331465
- [4]Perissinotti AJ, Bishop MR, Bubalo J Expert consensus guidelines for the prophylaxis and management of tumor lysis syndrome in the United States: Results of a modified Delphi panel Cancer Treat Rev, 2023.PMID 37579533
- [5]Prusakowski MK, Cannone D Pediatric Oncologic Emergencies Hematol Oncol Clin North Am, 2017.PMID 29078932
- [7]Quraishi NA, Palliyil N, Hassanin MA Malignant spinal cord compression in the paediatric population-a systematic review, meta-analysis. Eur Spine J, 2023.PMID 37338630
- [9]Hammami MB, Qasim A, Thakur R, et al. Rasburicase-induced hemolytic anemia and methemoglobinemia: a systematic review of current reports Ann Hematol, 2024.PMID 37468669