Paeds · haematology-oncology-and-transfusion
Tumour lysis syndrome and oncologic emergencies
Also known as Tumour lysis syndrome · Oncologic emergencies · Cairo-Bishop syndrome · Superior vena cava syndrome · Spinal cord compression · Hyperleukocytosis · Febrile neutropenia
Fellowship guide to tumour lysis syndrome and the paediatric oncologic emergencies. Covers the Cairo-Bishop classification of laboratory and clinical tumour lysis syndrome with its thresholds for urate, potassium, phosphate and calcium, the pathophysiology of the metabolic cascade from tumour breakdown to acute kidney injury and arrhythmia, the prevention with hyperhydration and rasburicase with the glucose-six-phosphate-dehydrogenase contraindication, the management of the hyperkalaemia and the acute kidney injury, and the structural and haematological emergencies of febrile neutropenia, hyperleukocytosis and leukostasis, superior vena cava obstruction from an anterior mediastinal mass, and malignant spinal cord compression.
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- Tumour lysis syndrome declares within hours of starting chemotherapy and kills through hyperkalaemic arrhythmia and acute kidney injury; begin hyperhydration and rasburicase before the first dose for the high-risk child
- Rasburicase is contraindicated in glucose-six-phosphate-dehydrogenase deficiency because it causes severe haemolysis and methaemoglobinaemia; check the status in the at-risk child
- A child with a white cell count over one hundred times ten to the nine per litre risks leukostasis in the brain and lungs; hydrate and cytoreduce, and do not transfuse red cells first because it worsens the viscosity
- An anterior mediastinal mass with stridor or superior vena cava obstruction is an anaesthetic catastrophe; never sedate before the airway is secured and keep the child upright
- Back pain with leg weakness or bowel or bladder change in a child with cancer is malignant spinal cord compression until proven otherwise; give dexamethasone and arrange urgent whole-spine magnetic resonance imaging within hours
- Fever in a child with a neutrophil count under zero point five times ten to the nine per litre is febrile neutropenia; give an antipseudomonal beta-lactam within one hour of presentation after blood cultures
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- Names tumour lysis syndrome as a metabolic emergency of rapid tumour breakdown defined by the Cairo-Bishop criteria of hyperkalaemia, hyperphosphataemia, hyperuricaemia and hypocalcaemia
- Recognises the paediatric oncologic emergencies of febrile neutropenia, hyperleukocytosis, superior vena cava obstruction and spinal cord compression as time-critical presentations
- States that fever in a child with a neutrophil count under zero point five times ten to the nine per litre is febrile neutropenia needing an antipseudomonal beta-lactam within one hour
- Explains how the rapid release of intracellular potassium, phosphate and urate from a bulky tumour overwhelms renal clearance and drives acute kidney injury, arrhythmia and seizure
- Distinguishes laboratory from clinical tumour lysis syndrome by the Cairo-Bishop criteria and applies the risk stratification to choose rasburicase over allopurinol
- Coordinates the multidisciplinary management of the oncologic emergencies across paediatric oncology, intensive care, nephrology, anaesthesia and neurosurgery
- Cairo-Bishop laboratory tumour lysis syndrome requires two or more of urate over four hundred and seventy-six micromoles per litre, potassium over six millimoles per litre, phosphate over two point one millimoles per litre in the child, and corrected calcium under one point seven five millimoles per litre, within three days before to seven days after cytotoxic therapy
- Tumour lysis prophylaxis for the high-risk child uses hyperhydration with isotonic potassium-free fluid at two to three litres per square metre per day, rasburicase at zero point one five to zero point two milligrams per kilogram, and four-to-six-hourly biochemistry
- A white cell count over one hundred times ten to the nine per litre risks leukostasis and is managed by hydration and cytoreduction without an early red-cell transfusion
- Synthesises the recognition, prevention and stepwise management of tumour lysis syndrome and the four oncologic emergencies into a single bedside plan in the specialist centre
- Counsels the family of a child at high risk of tumour lysis on the rationale for rasburicase, the need for close biochemical monitoring, and the glucose-six-phosphate-dehydrogenase check
- Builds the disposition and retrieval plan for the unstable child with an oncologic emergency, with the airway-first principle for the superior vena cava obstruction and the urgent imaging and steroid for the cord compression
- Level 1: Recognition of the metabolic and structural oncologic emergencies in the child with cancer and the urgent referral pathway
- Level 2: Application of the Cairo-Bishop criteria, the risk stratification and the prophylaxis with hyperhydration and rasburicase
- Level 3: Coordination of the definitive management of tumour lysis syndrome and the other oncologic emergencies with the multidisciplinary team
- The Cairo-Bishop classification of tumour lysis syndrome into the laboratory and the clinical forms and the four biochemical derangements
- The pathophysiology of the metabolic cascade from tumour breakdown to acute kidney injury, arrhythmia and seizure
- The management of febrile neutropenia, hyperleukocytosis, superior vena cava obstruction and spinal cord compression in the child with cancer
- Structured assessment of the child with a suspected oncologic emergency for the metabolic, haematological and structural threats
- Recognition that an anterior mediastinal mass with stridor or superior vena cava obstruction is an anaesthetic catastrophe and that no sedation is given before the airway is secured
- Communication of the plan for tumour lysis prophylaxis and the oncologic emergencies to the child and family
- Tumour lysis syndrome as the metabolic emergency of rapidly proliferating tumours and the Cairo-Bishop criteria for its diagnosis
- The high-risk tumours for tumour lysis including Burkitt lymphoma, lymphoblastic lymphoma, T-cell acute lymphoblastic leukaemia and acute myeloid leukaemia with a high white cell count
- The role of rasburicase and the contraindication in the glucose-six-phosphate-dehydrogenase deficiency
- Recognition of the time-critical oncologic emergencies in the child with cancer and the initial stabilisation in the referring hospital
- Application of tumour lysis prophylaxis with hyperhydration and rasburicase before the first dose of chemotherapy for the high-risk child
- Coordination of the diagnostic and therapeutic pathway and the transfer to the specialist paediatric oncology centre
- Canadian approach to the recognition and prophylaxis of tumour lysis syndrome in the high-risk child
- The Cairo-Bishop criteria and the risk stratification that guides the choice of rasburicase over allopurinol
- The management of the structural oncologic emergencies of superior vena cava obstruction and spinal cord compression
Overview & Definition
A child with a large, fast-growing tumour sits on the edge of a metabolic cliff. The moment chemotherapy or steroids begin to kill the tumour cells, those cells spill their contents into the blood faster than the kidneys can clear them, and within hours the potassium can stop the heart and the phosphate and urate can shut the kidneys down. This is tumour lysis syndrome, and it is one of the few complications of cancer treatment that can kill a child in the first day of therapy. The whole point of recognising it is to stand on the cliff edge first: to anticipate it before the first dose of chemotherapy, to flush the kidneys hard, and to dissolve the urate with rasburicase, so that the child never falls over. [1]
Tumour lysis syndrome is the name for the cluster of metabolic derangements that follow the rapid breakdown of a bulky tumour. The dying cells release their intracellular potassium, phosphate and nucleic acids, and the nucleic acids are turned into urate. When the release outstrips the renal excretion, potassium rises, phosphate rises, urate rises, and the calcium falls, and the child develops an acute kidney injury, a cardiac arrhythmia, or a seizure. The classification that every candidate must carry is the Cairo-Bishop definition, which separates the laboratory tumour lysis syndrome, a pattern on the blood test, from the clinical tumour lysis syndrome, the same pattern plus an organ that fails. [2]
This topic holds tumour lysis at its centre because it is the model oncologic emergency: the danger is inside the child, it is predictable, and it is preventable. Around it sit the other paediatric oncologic emergencies that share the same logic of anticipation and speed. Fever in the neutropenic child is a bacterial emergency that kills within hours, so the antibiotic is given within one hour of arrival. A white cell count over one hundred times ten to the nine per litre makes the blood too thick to flow through the brain and the lungs, so the cells are lowered before the blood is transfused. An anterior mediastinal mass can collapse airway moment sedation removes the tone that holds it open, so the airway is secured before any procedure. A tumour pressing on the spinal cord can paralyse a child within a day, so the steroid and the scan are given without delay. Each of these is a decision that changes an outcome, which is why they sit together in the fellowship examination. [5][6]
References11ShowHide
- [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
- [6]Briggs JE, Rabin M Oncologic Emergencies Pediatr Rev, 2026.PMID 42219190
- [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]Lowe EJ, Pui CH, Hancock ML, et al. Early complications in children with acute lymphoblastic leukemia presenting with hyperleukocytosis Pediatr Blood Cancer, 2005.PMID 15547931
- [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
- [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
- [11]Leung KKY, Hon KL, Hui WF, et al. Therapeutics for paediatric oncological emergencies Drugs Context, 2021.PMID 34234831