Lymphoblastic predominance of blastic phase in children with chronic myeloid leukaemia treated with imatinib: A report from the I-CML-Ped Study
Jazyk angličtina Země Velká Británie, Anglie Médium print-electronic
Typ dokumentu časopisecké články
PubMed
32799036
DOI
10.1016/j.ejca.2020.06.024
PII: S0959-8049(20)30357-9
Knihovny.cz E-zdroje
- Klíčová slova
- Accelerated phase, Blastic phase, Children, Chronic myeloid leukaemia, Haematopoietic stem cell transplantation, Tyrosine kinase inhibitors,
- MeSH
- blastická krize patologie MeSH
- chronická myeloidní leukemie farmakoterapie MeSH
- dítě MeSH
- imatinib mesylát terapeutické užití MeSH
- kojenec MeSH
- lidé MeSH
- lymfocyty metabolismus MeSH
- mladiství MeSH
- předškolní dítě MeSH
- Check Tag
- dítě MeSH
- kojenec MeSH
- lidé MeSH
- mladiství MeSH
- mužské pohlaví MeSH
- předškolní dítě MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- Názvy látek
- imatinib mesylát MeSH
BACKGROUND: Chronic myeloid leukaemia (CML) is a rare disease in children. The frequency and outcome of children evolving to accelerated phase (AP) or blastic phase (BP) under treatment with imatinib is unknown. The aim of the current study is to assess the incidence of progression from CML in chronic phase with imatinib frontline in a paediatric setting and describe the management and outcome of these patients. PATIENTS AND METHODS: In the I-CML-Ped Study database (www.clinicaltrials.gov, #NCT01281735), 19 of 339 paediatric patients in chronic phase treated with imatinib in the frontline evolved to CML-AP or CML-BP. RESULTS: With a median follow-up of 38 months (range: 2-190 months), the cumulative incidence of progression at 1 and 3 years was 3% (confidence interval [CI] 95%: 1-5%) and 7% (CI 95%: 4-11%), respectively. We observed a large predominance of lymphoid-BP (70%) over myeloid-BP (30%) with imatinib in frontline therapy. Sixteen patients underwent haematopoietic stem cell transplantation, and eight were treated with a tyrosine kinase inhibitor after transplant. Only the transplanted patients are alive. The 5-year overall survival rate of children with CML-AP/BP is 44%, with no statistical difference between the lymphoid-BP and myeloid-BP outcome. CONCLUSION: Children evolving to AP or BP under treatment with imatinib have a very poor prognosis with an overall survival under 50%, much worse than children with advanced phase at diagnosis.
Department of Pediatric Hemato Oncology University Hospital Motol Prague Czech Republic
Department of Pediatric Hematology Armand Trousseau Hospital APHP Sorbonne Université Paris France
Department of Pediatric Hematology Oncology and Transplantation Wroclaw Medical University Poland
Department of Pediatric Hematology Robert Debré Hospital APHP Université de Paris Paris France
Department of Pediatrics Ghent University Hospital Belgium
Department of Pediatrics Prince of Wales Hospital Hong Kong China
Department of Pediatrics Rigshospitalet University Hospital Copenhagen Denmark
Inserm CIC 1402 University Hospital Poitiers France
Medical Faculty Pediatric Hemato Oncology Technical University Dresden Germany
Citace poskytuje Crossref.org
ClinicalTrials.gov
NCT01281735
