Prolonged fever, lower monocyte count at admission, higher CRP levels between the fifth and the eighth hospitalization days, increment trends of monocyte and platelet levels, and CRP level decrement were associated with BSI. These factors may serve as markers for BSI in pediatric oncology patients with FN.

ObjectivePegylated IFN‐α2a has been reported in two case reports as being efficacious in treating CDA‐I patients. This study aims to assess its efficacy on a series of CDA‐I patients.MethodsStudy sample consisted of seven CDA type 1 transfusion‐dependent patients. They received pegylated interferon alpha‐2a at an initial dose of 90‐180 µg once a week, tapered according to clinical response and side effects. Good response was defined as Hb ≥ 10 g/dL for ≥3 months, partial response was defined as 7 ≤ Hb<10 g/dL for ≥3 months, and no response was defined as HB < 7 g/dL for over 3 months on treatment. Time to response was defined as the time needed to achieve hemoglobin levels ≥ 10 g/dL without transfusion. Patients were evaluated periodically by abdominal ultrasounds to rule out liver adenomas.ResultsFive patients (71%) had a good response to treatment. One patient stopped treatment due to side effects. One patient had partial response. One patient, with more severe phenotype and poor compliance, had poor response to treatment. No abnormal findings were found in ultrasound examination. No effect on serum ferritin level could be established.ConclusionPegylated interferon α2a therapy is efficacious in CDA‐I patients with a reasonable safety profile.

High grade gliomas (HGG) in children carry a dismal prognosis. Standard therapy includes resection when possible, radiotherapy and sometimes the addition of temozolomide. There is no standard treatment for progression or relapse. Since November 2018 we have offered upfront molecular testing to all children with HGG who had biopsy/ resection. Testing was mainly done by next generation sequencing panel, and some had research based methylation profiling and RNA seq. We aimed to see whether families chose to receive additional investigational treatment as a result of the molecular testing results and whether the treatment involved participation in a clinical study, or whether treatment was compassionate. A total of 22 patients aged 2.8-16.8 years with HGG had a biopsy/resection over this three year period. Thirteen had diffuse midline glioma (DMG) of which 11 had the H3K27 mutation, and 9 were cortical. Six children had underlying predisposition syndromes: mismatch repair deficiency (n=3 proven + 1 highly suspected), neurofibromatosis1 (n=1), Li-Fraumeni (n=1). All the cortical gliomas had potential treatment options based on their molecular testing. 10/22 (45%) children received investigational therapy of which only three participated in a clinical study while the rest received compassionate therapy. Compassionate treatments included BRAF/MEK inhibitors (n=4), Larotrectinib (n=1), and immune checkpoint inhibitors (n=2). Of the 12 who did not receive investigational therapy, four, all cortical, have potential therapy options but are currently in remission. Of the remaining eight, two had very rapid clinical deterioration and died, and six (all DMG) did not wish/ were unable to travel abroad and no relevant clinical study was available locally. We conclude that the families of children with HGG are highly motivated to receive investigational therapy. Upfront molecular testing of these tumors, especially for cortical HGG, is imperative and there is a growing need for accessible clinical studies.

Constitutional symptoms and pancytopenia are occasionally the initial presentation of pediatric brucellosis. Therefore, in endemic areas, in children with pancytopenia, both brucellosis and malignancy should be included in the deferential diagnosis. We report here a child with pancytopenia and hepatosplenomegaly as manifestations of brucellosis in whom bone marrow morphology and flow cytometry data revealed hemophagocytosis, left shift in myeloid cells and activation changes in antigenic properties of T and B lymphocytes and monocytes. The patient had an uneventful and complete recovery after appropriate antibiotic therapy. Our report demonstrates that bone marrow and flow cytometry findings in children with brucellosis may include significant reactive changes in hematopoiesis.