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Case history

image A 2-year-old boy presented with increasing lethargy, bruising and epistaxis, and 4 months of intermittent coryzal symptoms and abdominal swelling. He had been born by normal delivery. He was up to date with his immunizations and had normal developmental progress. He had no known drug allergies. He was an only child and attended nursery 2 days per week.

On examination, he was afebrile, lethargic, pale and miserable. He had multiple bruises on his head, trunk and limbs. He had marked hepatosplenomegaly extending below the umbilicus, and cervical and occipital lymphadenopathy. He had no focal neurological signs, normal tone and down-going plantars.

Investigations revealed haemoglobin 30 g/l, white blood cell count 1093×109/l, platelets 9×109/l and blood film showing 74% lymphoblasts consistent with acute lymphoblastic leukaemia (ALL). Immunophenotyping performed on peripheral blood demonstrated a large population of cluster of differentiation (CD)-positive blasts: CD3, CD4, CD7, CD34, CD2, terminal deoxynucleotidyl transferase, CD5, partial CD117 and partial CD13, confirming a diagnosis of T cell ALL. Cytogenetic analysis of peripheral blood revealed two abnormal clones with 6q loss. Loss of 6q is a general marker of lymphoid neoplasia and is frequently seen in T cell disease; it has no significant effect on prognosis.

The patient was transferred to intensive care, where initial management involved hyperhydration, blood titration, an exchange transfusion and dexamethasone. He did not develop tumour lysis or leucostasis. His parents consented to his recruitment to the United Kingdom Trial for Children and Young Adults with Acute Lymphoblastic Leukaemia and Lymphoma (UKALL) 2011. As he had a diagnosis of T lineage ALL, he commenced regimen B chemotherapy and was randomized to receive short course dexamethasone during induction.

Day 29 (end of induction) bone marrow aspirate revealed a hypocellular marrow with regeneration of erythroid cells and 4% blasts consistent with morphological remission. Polymerase chain reaction (PCR) to quantify minimal residual disease (MRD) status was reported as 'risk' (≥0.005% leukaemia cells). He was therefore transferred to regimen C treatment, as per the protocol. Bone marrow aspiration at week 14 of treatment was reported as MRD 'intermediate risk' (≤0.5% leukaemia cells), and he therefore continued on this arm of therapy.

What are the signs and symptoms of leucostasis and principles of initial management?

What is tumour lysis syndrome and what can be done to reduce the risk of tumour lysis in high-count leukaemia?

What are the overarching principles of UKALL 2011?

How is MRD response incorporated into the management of childhood ALL?

What late effects can this child and his family anticipate?

What are the signs and symptoms of leucostasis and principles of initial management?

Hyperleucocytosis occurs in 10–20% of patients with ALL.1 There is a particular risk of acute morbidity and mortality in ALL and acute myeloid leukaemia when the white blood cell count exceeds 100×109/l.2 Leucostasis is diagnosed clinically when a patient with hyperleucocytosis develops respiratory or neurological distress ...

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