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Paeds Vivashaematology-oncology-and-transfusion

Paeds Vivas · haematology-oncology-and-transfusion

Tumour lysis syndrome and oncologic emergencies: Viva

Branching clinical structured oral on tumour lysis syndrome and the paediatric oncologic emergencies, covering the Cairo-Bishop classification of the laboratory and the clinical syndrome with the thresholds for the urate, the potassium, the phosphate and the calcium, the pathophysiology of the metabolic cascade to the acute kidney injury and the arrhythmia, the prevention with the hyperhydration and the rasburicase with the glucose-six-phosphate-dehydrogenase contraindication, the management of the hyperkalaemia and the acute kidney injury, the hyperleukocytosis and the leukostasis, the febrile neutropenia, the superior vena cava obstruction from the anterior mediastinal mass, and the malignant spinal cord compression.

branching clinical structured oral5 min readVerification in progress

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Prompt
A seven-year-old boy presents with a large abdominal mass, a lactate dehydrogenase of three thousand, and a biopsy confirming the Burkitt lymphoma, and he is about to start his first cycle of chemotherapy. The examiner asks how you assess his tumour lysis risk, what prophylaxis you put in place before the first dose, how you would recognise and manage the tumour lysis syndrome if it declares, and how your management would change if he also had an anterior mediastinal mass or a progressive leg weakness.

This is a branching oral built to probe the reasoning that holds the prevention of the tumour lysis at the centre, and to expose the candidate who has memorised the headline without the corners. The questions escalate from the risk assessment to the prophylaxis, the recognition, the management, and the structural emergencies, with the deliberate probes into the pitfalls. [1]

Opening question: the risk and the prophylaxis

The examiner opens with the Burkitt lymphoma and asks how you assess his tumour lysis risk and what prophylaxis you put in place before the first dose. [3]

A strong answer names the Burkitt lymphoma as the very highest-risk tumour, with its rapid doubling time of around twenty-four hours and its large mass, and the high lactate dehydrogenase confirming the high burden. The prophylaxis is the hyperhydration with an isotonic potassium-free fluid at two to three litres per square metre per day to keep the urine output above two millilitres per kilogram per hour, the rasburicase at zero point one five to zero point two milligrams per kilogram, and the four-to-six-hourly biochemistry for the first twenty-four to forty-eight hours. [1]

Model answer. This boy is at the very top of the tumour lysis risk because the Burkitt lymphoma has the highest cell turnover of any childhood tumour. I would start the hyperhydration with an isotonic potassium-free fluid, give the rasburicase at zero point one five to zero point two milligrams per kilogram after checking the glucose-six-phosphate-dehydrogenase status, and monitor the biochemistry every four to six hours, with the renal replacement therapy on standby. [1][4]

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References8ShowHide
  1. [1]Howard SC, Avagyan A, Workeneh B, Pui CH Tumour lysis syndrome Nat Rev Dis Primers, 2024.PMID 39174582
  2. [2]Cairo MS, Bishop M Tumour lysis syndrome: new therapeutic strategies and classification Br J Haematol, 2004.PMID 15384972
  3. [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. [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. [10]Lehrnbecher T, Robinson PD, Ammann RA, et al Guideline for the Management of Fever and Neutropenia in Pediatric Patients With Cancer and Hematopoietic Cell Transplantation Recipients: 2023 Update. J Clin Oncol, 2023.PMID 36689694
  6. [5]Prusakowski MK, Cannone D Pediatric Oncologic Emergencies Hematol Oncol Clin North Am, 2017.PMID 29078932
  7. [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
  8. [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
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