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Paeds Vivashaematology-oncology-and-transfusion

Paeds Vivas · haematology-oncology-and-transfusion

Leukaemia in children: Viva

Branching clinical structured oral on leukaemia in children, covering the recognition of the red-flag presentation, the resuscitation with irradiated leucodepleted transfusion, tumour lysis prophylaxis with rasburicase and empiric antipseudomonal cover, the diagnostic pathway with flow cytometry and cytogenetics, the risk stratification by age, white cell count, genetics and minimal residual disease, the risk-adapted therapy, and the special scenarios of Down syndrome, the infant, the Philadelphia positive disease and the relapse.

branching clinical structured oral4 min readVerification in progress

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RACP DWERACP DCEMRCPCH Clinical
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RACP DWERACP DCEMRCPCH Clinical
Prompt
A four-year-old boy is brought in pale, covered in bruises, with a three-week history of fatigue and a fever. His full blood count shows haemoglobin 62 g per litre, a white cell count of 35 times ten to the nine per litre, neutrophils 0.4 times ten to the nine per litre, platelets 22 times ten to the nine per litre, and the peripheral film shows lymphoblasts. The examiner asks how you frame the problem, what you do in the first hours, how you confirm the diagnosis and assign the risk, and how you counsel the family.

This is a branching oral built to probe the reasoning that holds the risk stratification and the resuscitation-before-diagnosis at the centre, and to expose the candidate who has memorised the headline without the corners. The questions escalate from the framing to the stabilisation, the diagnosis, the risk assignment, and the definitive therapy, with deliberate probes into the pitfalls. [1]

Opening question: framing the problem

The examiner opens with the film and the count and asks how you frame this problem in a single sentence, and what your first priority is. [9]

A strong answer names acute leukaemia with the circulating lymphoblasts, and states that the first priority is the resuscitation of the dangerous elements, the anaemia, the bleeding risk, the neutropenic fever, and the tumour lysis risk. [7]

Model answer. This child has a trilineage cytopenia with circulating lymphoblasts, which is acute lymphoblastic leukaemia until proven otherwise. My first priority is to resuscitate the dangerous elements, and then to confirm the diagnosis with an urgent bone marrow aspirate and trephine biopsy in a specialist centre. [1]

Probe one: the resuscitation

The examiner presses for exactly what you do in the first four hours, and why you choose those blood products. [7]

A strong answer reproduces the three legs. Red cells are transfused for the symptomatic anaemia, given slowly, and all the cellular products are irradiated and leucodepleted to prevent the transfusion-associated graft-versus-host disease. Platelets are held for a count under ten times ten to the nine per litre in the stable child and under twenty in the febrile or the bleeding child. Tumour lysis is prevented with the hyperhydration using an isotonic fluid without potassium, the rasburicase for the high-risk child, and the four-to-six-hourly biochemistry. The febrile neutropenia gets an empiric antipseudomonal beta-lactam within one hour after the blood cultures. [3][7]

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References6ShowHide
  1. [1]Hunger SP, Mullighan CG Acute Lymphoblastic Leukemia in Children N Engl J Med, 2015.PMID 26465987
  2. [3]Howard SC, Avagyan A, Workeneh B Tumour lysis syndrome Nat Rev Dis Primers, 2024.PMID 39174582
  3. [6]Verma A, Lupo PJ, Shah NN Management of Down Syndrome-Associated Leukemias: A Review JAMA Oncol, 2023.PMID 37440251
  4. [7]Prusakowski MK, Cannone D Pediatric Oncologic Emergencies Hematol Oncol Clin North Am, 2017.PMID 29078932
  5. [9]Fragkandrea I, Nixon JA, Panagopoulou P Signs and symptoms of childhood cancer: a guide for early recognition Am Fam Physician, 2013.PMID 23939697
  6. [10]Bhojwani D, Pui CH Relapsed childhood acute lymphoblastic leukaemia Lancet Oncol, 2013.PMID 23639321
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