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Paeds Caseshaematology-oncology-and-transfusion

Paeds Cases · haematology-oncology-and-transfusion

Leukaemia in children: Case

Clinical long case of a four-year-old boy presenting with pallor, bruising and fever and a trilineage cytopenia with circulating lymphoblasts, covering the recognition of the emergency, the resuscitation with irradiated leucodepleted red cell and platelet transfusion, the tumour lysis prophylaxis with hyperhydration and rasburicase, the empiric antipseudomonal cover for the febrile neutropenia, the diagnostic pathway with flow cytometry and cytogenetics, the risk stratification and the risk-adapted therapy, and the family counselling.

paediatric haematology long case4 min readVerification in progress

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RACP DCEMRCPCH ClinicalRCPSC Pediatrics
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RACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
A four-year-old boy is brought to the emergency department by his parents with a three-week history of increasing pallor and tiredness, easy bruising over the limbs, and intermittent fever. His mother has noticed he no longer runs around at preschool and that the bruises appeared without injury. On examination he is pale, with petechiae over the lower limbs and shins, palpable cervical and axillary lymph nodes, and a liver and spleen palpable three and four centimetres below the costal margins. 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, and platelets 22 times ten to the nine per litre, and the peripheral film shows circulating lymphoblasts. The examiner asks how you frame the problem, how you stabilise and confirm the diagnosis, how you assign the risk and plan the therapy, and how you counsel the family.

Framing the case

This four-year-old boy has the classic presentation of acute lymphoblastic leukaemia. The trilineage cytopenia, the circulating lymphoblasts, the organomegaly and the lymphadenopathy, alongside the three-week history of the pallor, the bruising and the fever, together make a marrow malignancy the working diagnosis, and the first decision is to move the child from an elective workup to an emergency resuscitation. The framework that organises the case is the recognition that this is a clonal lymphoid malignancy, and the first priority is the resuscitation before the diagnosis is pursued. [9][1]

Immediate assessment and stabilisation

The assessment begins with the airway, the breathing and the circulation, because a child with a severe anaemia, a bleeding risk and a fever is in danger before any diagnosis is reached. The child is admitted to a paediatric haematology-oncology centre, and the resuscitation rests on the three legs. Red cells are transfused for the symptomatic anaemia, given slowly to avoid the circulatory overload, with all the cellular products irradiated and leucodepleted to prevent the transfusion-associated graft-versus-host disease. Platelets are transfused for the count under twenty times ten to the nine per litre in this febrile child. [7]

Tumour lysis prophylaxis begins before the first chemotherapy dose, with the hyperhydration using an isotonic fluid without potassium, the rasburicase for the high-risk child, and the four-to-six-hourly biochemistry of the potassium, the phosphate, the calcium, the creatinine and the urate. The glucose-6-phosphate dehydrogenase status is checked before the rasburicase because it is contraindicated in the deficiency. The blood cultures are drawn and an empiric antipseudomonal beta-lactam such as the piperacillin-tazobactam is given within one hour for the febrile neutropenia, with a neutrophil count under zero point five times ten to the nine per litre. [3][7]

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References5ShowHide
  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. [5]Cooper SL, Brown PA Treatment of pediatric acute lymphoblastic leukemia Pediatr Clin North Am, 2015.PMID 25435112
  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
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