Leukaemia in children: Case

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]

The diagnostic pathway and the risk assignment

The bone marrow aspirate and the trephine biopsy are performed together at the posterior iliac crest once the child is stable. The aspirate provides the cells for the morphology, for the flow cytometry to define the B-cell lineage by the CD19 and CD10, and for the cytogenetics and the molecular panel. The diagnosis rests on the integration of the morphology, the immunophenotype and the genetics, and the lumbar puncture with the intrathecal therapy is performed at the specialist centre. The standard risk, by the clinical criteria, is the age of one to under ten years with a white cell count under fifty times ten to the nine per litre, and this boy at four years with a count of thirty-five is the standard risk. [1]

The named diagnosis and the definitive management

The bone marrow confirms the B-cell precursor acute lymphoblastic leukaemia, the standard risk by the age and the initial white cell count, with a favourable cytogenetic finding of the ETV6-RUNX1 fusion. The definitive management is the risk-stratified multi-agent chemotherapy delivered over two and a half years through the phases of the remission induction, the consolidation, the interim maintenance, the delayed intensification, and the maintenance, with the central nervous system-directed therapy woven through every phase. The induction backbone includes the vincristine, the dexamethasone, the asparaginase and the daunorubicin, with the intrathecal therapy from day one, and it achieves the remission in over ninety-five percent within four weeks. [5]

The boy is enrolled on the national protocol, and the multidisciplinary team is assembled. The family is taught the neutropenic precautions, the fever as an emergency, the central line care, and the recognition of the late effects. The survivorship plan is begun from the day of the diagnosis, with the surveillance for the anthracycline cardiotoxicity, the endocrine late effects, and the second malignancy risk. [1][5]

Communication and the family

The family is counselled honestly and hopefully. The candidate names the diagnosis, explains that it is the commonest childhood cancer, that the contemporary survival exceeds ninety percent on the modern protocols, and that the treatment runs over two to three years. The parents are introduced to the multidisciplinary team, given a written plan, taught the fever and the bleeding emergencies, and supported by the social work and the educational liaison. The child is prepared in an age-appropriate way, and the siblings and the school are included. [9]