Paeds · haematology-oncology-and-transfusion
Leukaemia in children
Also known as Acute lymphoblastic leukaemia · Acute myeloid leukaemia · Childhood leukaemia · ALL · AML · Tumour lysis syndrome
Fellowship guide to leukaemia in children. Covers the separation of acute lymphoblastic from acute myeloid leukaemia, the peak age of two to five years, the presentation of pallor, bruising, fever and bone pain with circulating blasts, the recognition and prevention of tumour lysis syndrome with hyperhydration and rasburicase, the risk stratification by age, white cell count, cytogenetics and minimal residual disease, the risk-adapted multi-agent chemotherapy through induction, consolidation and maintenance, the central nervous system-directed therapy, the Down syndrome transient myeloproliferative disorder and myeloid leukaemia, and the oncologic emergencies of hyperleukocytosis, mediastinal mass and febrile neutropenia.
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Target exams
Red flags
- A pale, bruising, febrile child with circulating blasts has leukaemia until the bone marrow proves otherwise
- A white cell count over one hundred times ten to the nine per litre risks leukostasis in the brain and lungs and needs urgent cytoreduction before any red cell transfusion
- An anterior mediastinal mass with stridor or superior vena cava obstruction is an anaesthetic catastrophe; never sedate before the airway is secured
- Acute promyelocytic leukaemia carries a high early mortality from disseminated intravascular coagulation; start all-trans retinoic acid on suspicion, not after confirmation
- Tumour lysis syndrome declares within hours of starting chemotherapy and kills through hyperkalaemia and acute kidney injury; begin hyperhydration and rasburicase before the first dose
- 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
Life stages
Care settings
Clinical exam formats
Board mappings
- Names acute lymphoblastic leukaemia as the commonest childhood cancer and separates it from acute myeloid leukaemia by lineage
- Recognises the red-flag presentation of a pale, bruising, febrile child with circulating blasts and arranges urgent referral to paediatric haematology-oncology
- States the tumour lysis syndrome triad of hyperkalaemia, hyperphosphataemia and hyperuricaemia and the need for prophylaxis before chemotherapy
- Explains how a clonal expansion of lymphoblasts or myeloblasts crowds the marrow, causes trilineage failure, and drives the oncologic emergencies of tumour lysis and hyperleukocytosis
- Coordinates the diagnostic pathway from full blood count and film through bone marrow aspirate, flow cytometry, cytogenetics and molecular testing to risk-stratified therapy
- Distinguishes B-cell precursor from T-cell acute lymphoblastic leukaemia, recognises the favourable and unfavourable cytogenetics, and applies the minimal residual disease result to the risk group
- Standard-risk acute lymphoblastic leukaemia is age one to under ten years and a white cell count under fifty times ten to the nine per litre
- Tumour lysis prophylaxis for a high-risk leukaemia uses hyperhydration with isotonic fluid without potassium, rasburicase or allopurinol, and four-to-six-hourly biochemistry
- Febrile neutropenia is treated empirically with an antipseudomonal beta-lactam within one hour of presentation after blood cultures
- Counsels the family of a child newly diagnosed with acute lymphoblastic leukaemia on the diagnosis, the two to three year treatment, and the contemporary survival of over ninety percent
- Builds the multidisciplinary plan linking paediatric oncology, infectious diseases, the diagnostic laboratory, the family and the primary care team
- Synthesises the long-case child with leukaemia into a treatment, supportive care and survivorship plan covering relapse surveillance and late effects
- Level 1: Recognition of the red-flag features of childhood leukaemia on the full blood count and film and the urgent referral pathway
- Level 2: Application of the bone marrow aspirate, flow cytometry and cytogenetics to reach the named diagnosis and risk group
- Level 3: Coordination of risk-adapted therapy, central nervous system prophylaxis, supportive care and long-term survivorship
- The classification of childhood leukaemia into B-cell precursor and T-cell acute lymphoblastic leukaemia and acute myeloid leukaemia
- The favourable cytogenetics of ETV6-RUNX1 and high hyperdiploidy and the unfavourable KMT2A rearrangement, hypodiploidy and BCR-ABL1
- The pathophysiology and prevention of tumour lysis syndrome and the management of febrile neutropenia
- Structured assessment of the pale, bruising child for pallor, petechiae, organomegaly, lymphadenopathy and a mediastinal mass
- Communication of a new diagnosis of leukaemia to a child and family with honesty, hope and a clear plan
- Recognition of the oncologic emergencies of hyperleukocytosis, tumour lysis syndrome, mediastinal mass and febrile neutropenia
- Childhood leukaemia as the commonest malignancy of childhood and the basis for the early recognition of the red-flag presentation
- The diagnostic roles of the peripheral film, bone marrow aspirate, flow cytometry, cytogenetics and molecular testing
- Down syndrome as a predisposition to the transient myeloproliferative disorder and to both lymphoblastic and myeloid leukaemia
- Recognition of the red-flag clinical and laboratory features of childhood leukaemia that mandate urgent referral
- Application of the initial stabilisation including transfusion of irradiated leucodepleted products, tumour lysis prophylaxis and empiric antibiotics
- Coordination of the diagnostic and multidisciplinary pathway and the transition into risk-adapted therapy
- Canadian approach to the urgent evaluation and referral of a child with newly suspected leukaemia
- Bone marrow aspirate with flow cytometry and cytogenetics as the diagnostic standard and the basis of risk stratification
- The contemporary risk-adapted protocols and the role of minimal residual disease in guiding therapy
Overview & Definition
A child is brought in pale, covered in bruises, with a fever that will not settle, and the blood film shows blasts. This is leukaemia until the bone marrow proves otherwise, and the first task at the bedside is to recognise the emergency and to move the child from an elective workup into a resuscitation. Leukaemia is the commonest malignancy of childhood, and the acuity of the presentation, with its threat of bleeding, sepsis and the metabolic disaster of tumour lysis, is what makes the first hours decisive. A delay of days can change the outcome, which is why every paediatrician must hold the diagnosis actively in mind. [9]
Leukaemia is a clonal malignancy of the haematopoietic precursor cell in which the normal maturation of a single lineage is arrested and the immature blast accumulates in the bone marrow. The accumulation crowds out the normal red cell, white cell and platelet precursors, producing the trilineage failure that explains almost every symptom, and the blasts spill into the peripheral blood. The disease is defined by the lineage of the arrested cell, and the separation into the lymphoid and the myeloid forms is the single most important early distinction because it determines the chemotherapy, the prognosis and the supportive care. [1]
The acute leukaemias dominate childhood, and of these acute lymphoblastic leukaemia is by far the commonest, accounting for roughly three quarters to four fifths of all childhood leukaemia. Acute myeloid leukaemia is less common but more evenly spread across the paediatric age range, and it carries a distinctive neonatal presentation in the infant with Down syndrome. The chronic leukaemias are rare in children and fall outside the everyday paediatric encounter, which is why this topic is built around the two acute forms and the emergencies they bring. [1]
The gravity of the diagnosis, and the remarkable improvement in its outlook, are why the topic sits at the centre of the fellowship examination. A candidate who can recognise the presentation, read the count and the film, chart the urgent diagnostic pathway, anticipate and prevent the oncologic emergencies, and explain the risk-adapted therapy to a family is demonstrating exactly reasoning boards reward. The contemporary survival of childhood acute lymphoblastic leukaemia now exceeds ninety percent in the best protocols, and the candidate who carries that number, alongside the humility of the emergencies, has the framework for the whole topic. [1][5]
References10ShowHide
- [1]Hunger SP, Mullighan CG Acute Lymphoblastic Leukemia in Children N Engl J Med, 2015.PMID 26465987
- [2]Rubnitz JE, Kaspers GJL How I treat pediatric acute myeloid leukemia Blood, 2021.PMID 34115839
- [3]Howard SC, Avagyan A, Workeneh B Tumour lysis syndrome Nat Rev Dis Primers, 2024.PMID 39174582
- [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]Cooper SL, Brown PA Treatment of pediatric acute lymphoblastic leukemia Pediatr Clin North Am, 2015.PMID 25435112
- [6]Verma A, Lupo PJ, Shah NN Management of Down Syndrome-Associated Leukemias: A Review JAMA Oncol, 2023.PMID 37440251
- [7]Prusakowski MK, Cannone D Pediatric Oncologic Emergencies Hematol Oncol Clin North Am, 2017.PMID 29078932
- [8]Sas V, Blag C, Zaharie G Transient leukemia of Down syndrome Crit Rev Clin Lab Sci, 2019.PMID 31043105
- [9]Fragkandrea I, Nixon JA, Panagopoulou P Signs and symptoms of childhood cancer: a guide for early recognition Am Fam Physician, 2013.PMID 23939697
- [10]Bhojwani D, Pui CH Relapsed childhood acute lymphoblastic leukaemia Lancet Oncol, 2013.PMID 23639321