Within the International Registry of Childhood Chronic Myeloid Leukemia (CML), we identified 18 patients less than 18 years old at diagnosis of CML who were in the chronic phase and exhibiting a sustained deep molecular response (DMR) to imatinib defined as BCR-ABL1/ABL1 < 0.01% (MR4) for at least two years followed by discontinuation of imatinib. Before discontinuation, the median duration of imatinib was 73.2 months (range, 32–109) and the median duration of MR4 was 46.2 months (range, 23.9–98.6). Seven patients experienced loss of major molecular response (MMR) 4.1 months (range, 1.9–6.4) after stopping and so restarted imatinib. The median molecular follow-up after discontinuation was 51 months (range, 6–100) for the nine patients without molecular relapse. The molecular free remission rate was 61% (95% CI, 38–83%), 56% (95% CI, 33–79%) and 56% (95% CI, 33–79%) at 6, 12 and 36 months, respectively. Six of the seven children who experienced molecular relapse after discontinuation regained DMR (median, 4.7 months; range, 2.5–18) after restarting imatinib. No withdrawal syndrome was observed. In univariate analysis, age, sex, Sokal and ELTS scores, imatinib treatment and DMR durations before discontinuation had no influence on treatment free remission. These data suggest that imatinib can be safely discontinued in children with sustained MR4 for at least two years.
PURPOSE: The Assessing Doctors' Attitudes on Palliative Treatment study was conducted in 11 Eurasian countries to assess physician knowledge of and structural barriers to integration of palliative care into pediatric oncology. After publication, regional collaborators identified the need to disseminate country-specific study results locally and provide policy recommendations to inform stakeholders. METHODS: The Assessing Doctors' Attitudes on Palliative Treatment report was developed with Eurasian and St Jude pediatric palliative care and oncology experts to summarize study findings and deliver country-level data to local stakeholders. In parallel, an assessment was developed to explore how regional collaborators intend to use the report to improve local advocacy and dissemination of research findings. The country report and assessment were translated to English, Russian, and Mongolian. RESULTS: Country-specific two-page reports display study findings on pediatric palliative care education, access to pediatric palliative care services, and barriers to and timing of integration with cancer care, alongside clinical and policy recommendations. These reports were distributed to collaborators in 11 countries. Assessment results (N = 30) demonstrated that regional collaborators planned to distribute the report to institutional and government stakeholders, aiming to increase access to pediatric palliative care services (77%), establish a community-based palliative care network (70%), and increase opportunities for specialization (70%). CONCLUSION: We describe the development of an evidence-based advocacy tool to inform local health and education policy in Eurasia. This summary report of study findings, translated to local languages and adapted to a broader audience, is currently used to advocate for greater access and quality of palliative care for children with cancer. This work may serve as the basis for future dissemination efforts of scientific research.
19 December 2016 in Pediatric Hematology/Oncology and Immunopathology
Chronic myeloid leukemia (CML) is rare in children up to the age of 18 years; for this reason, there are no accurate criteria of monitoring and individualization of treatment in this population group. This review of literature presents some features of the manifestation, the development of CML, the tyrosine kinase inhibitors treatment response and results of allogeneic stem cell transplantation in children. In addition this paper presents published data on the incidence of side effects during long-term imatinib therapy.
24 April 2019 in Russian Journal of Pediatric Hematology and Oncology
Chronic myeloid leukemia (CML) in children is rare, less than 3 % of all cases of leukemia in pediatric practice. Along with the successes achieved in the treatment of CML with imatinib, it’s necessary to study of molecular factors in predicting resistance to therapy. According to the literature, about 30 % of adult patients with imatinib resistance have point mutations in the kinase domain of BCR-ABL1 gene. The number of reports about mutation spectrum of the BCR-ABL1 gene in children with resistant forms of CML is limited. This article describes the clinical case of secondary resistance to imatinib in a 15-year-old girl with the F359C mutation of BCR-ABL1 gene and a review of the literature.
13 May 2024 in Russian Journal of Pediatric Hematology and Oncology
Introduction. The article analyzes the incidence of secondary malignant tumors (SMT) after therapy of Hodgkin’s lymphoma (HL) in 508 patients aged 0–18 years in the Republic of Belarus.Materials and methods. Patients received program therapy under the DAL-HD-90(m) protocol. The observation period is from 10 months to 25 years. During the entire follow-up period, 27 secondary malignant tumors were diagnosed in 26 (4.1 %) patients: thyroid cancer – 11 (40.7 %), acute myeloid leukemia – 2 (7.4 %), stomach cancer – 2 (7.4 %), upper lobe bronchus cancer – 1 (3.7 %), parotid salivary gland carcinoma – 2 (7.4 %), breast cancer – 1 (3.7 %), hepatocellular liver cancer – 1 (3.7 %), soft tissue sarcoma – 2 (7.4 %), renal cell carcinoma – 1 (3.7 %), ovarian tumor – 1 (3.7 %), myelodysplastic syndrome – 1 (3.7 %), basal cell skin cancer – 2 (7.4 %). All cases of secondary hematological tumors were fatal.Results. It was found that solid SMT developed within the area of radiation exposure to organs and tissues. The risk of developing SMT in patients with HL increases with increasing follow-up time after treatment. The cumulative incidence of SMT was 3.4 ± 0.8 % after 10 years, 4.6 ± 1.0 % after 15 years, and 7.0 ± 1.6 % 20 years after the diagnosis of HL. The latent period of SMT development after HL was 8.4 years (from 5 months to 23 years).Conclusion. The data confirmed the need to optimize the therapy volume, reduce doses and indications for radiation therapy (RT), consider prognostic factors, introduce new RT methods, and use new treatment regimens for children with HL.