Unusual lymphoid malignancy and treatment response in two children with Down syndrome.

BACKGROUND: Lymphoid malignancies other than acute lymphoblastic leukemia (ALL) are rare in children with Down syndrome (DS). Information about the toxicity of chemotherapy and prognosis is largely derived from the experience of children with DS and ALL or children without DS. PROCEDURE: We describe the treatment and outcome of two unusual lymphoid malignancies in children with DS. One patient was diagnosed with Burkitt lymphoma (BL) and the second, after treatment for B precursor ALL, with T-cell EBV-positive proliferative disorder (LPD). RESULTS: BL was treated with standard doses of LMB group B therapy subsequently intensified to group C therapy, including high-dose methotrexate (HD-MTX, 3-8 g/m2 ). The patient did not experience excessive toxicity and remains in complete remission 13 months later. Despite presentation with disseminated disease the patient with T-cell EBV-positive LPD after treatment for B precursor ALL responded to dexamethasone and rituximab and remains in complete remission two years later. CONCLUSIONS: Upfront reduction of the high treatment intensity, which is associated with excellent survival outcomes in BL, may not be warranted in all children with DS. Response to therapy and prognosis of T-cell EBV-positive LPD in a patient with DS was not predicted by reported experience in the absence of DS.

Severe hypoglycaemia in paediatric oncology: characterisation and risk factors.

OBJECTIVES: Primary objective was to describe the cumulative incidence of severe hypoglycaemia in paediatric patients with cancer. Secondary objectives were to determine risk factors for severe hypoglycaemia and to describe its clinical course and management. METHODS: In this single institution retrospective study, for the cumulative incidence cohort, we included cancer diagnosis and hypoglycaemia episodes between June 2018 and November 2021. For the chart review cohort, we included cancer diagnosis January 2009-November 2021 and hypoglycaemia episodes June 2018-November 2021. RESULTS: There were 1237 cancer diagnoses and 142 patients with severe hypoglycaemia in the cumulative incidence cohort. Cumulative incidence at 6 months after cancer diagnosis was 9.4% (95% CI 7.7% to 11.0%). Severe hypoglycaemia incidence significantly increased over time (r=0.77, p=0.004). Independent risk factors were age at diagnosis (HR 0.88, 95% CI 0.85 to 0.91); acute lymphoblastic leukaemia (HR 3.06, 95% CI 2.19 to 4.29) and relapse (HR 9.54, 95% CI 3.83 to 23.76). There were 4672 cancer diagnoses and 267 episodes of severe hypoglycaemia in the chart review cohort. CONCLUSIONS: The cumulative incidence of severe hypoglycaemia 6 months after cancer diagnosis was 9.4%. Severe hypoglycaemia increased over time. Younger patients, those with acute lymphoblastic leukaemia and those with a history of disease relapse, were at higher risk of severe hypoglycaemia.