Pancytopenia and marrow infiltration: Case

Framing the case

This four-year-old boy has the classic presentation of acute leukaemia with marrow infiltration. The trilineage cytopenia, the circulating blasts, the organomegaly and the lymphadenopathy, alongside the three-week history of pallor, bruising and 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 empty-versus-full marrow distinction, and here the blasts and the organomegaly settle the marrow as full. ^[1][3]

Immediate assessment and stabilisation

The assessment begins with the airway, breathing and 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 three legs. Red cells are transfused for the symptomatic anaemia, given slowly to avoid circulatory overload, with all cellular products irradiated and leucodepleted to prevent 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. ^[12]

Tumour lysis prophylaxis begins before the first chemotherapy dose, with hyperhydration using an isotonic fluid without potassium, rasburicase for the high-risk child, and four-to-six-hourly biochemistry of the potassium, phosphate, calcium, creatinine and urate. The glucose-6-phosphate dehydrogenase status is checked before the rasburicase because it is contraindicated in the deficiency. Blood cultures are drawn and an empiric antipseudomonal beta-lactam such as piperacillin-tazobactam is given within one hour for the febrile neutropenia, with a neutrophil count under 0.5 times ten to the nine per litre. ^[12]