BACKGROUND: The outcome of infants with KMT2A-germline acute lymphoblastic leukaemia (ALL) is superior to that of infants with KMT2A-rearranged ALL but has been inferior to non-infant ALL patients. Here, we describe the outcome and prognostic factors for 167 infants with KMT2A-germline ALL enrolled in the Interfant-06 study. METHODS: Univariate analysis on prognostic factors (age, white blood cell count at diagnosis, prednisolone response and CD10 expression) was performed on KMT2A-germline infants in complete remission at the end of induction (EOI; n = 163). Bone marrow minimal residual disease (MRD) was measured in 73 patients by real-time quantitative polymerase chain reaction at various time points (EOI, n = 68; end of consolidation, n = 56; and before OCTADAD, n = 57). MRD results were classified as negative, intermediate (<5∗10-4), and high (≥5∗10-4). RESULTS: The 6-year event-free and overall survival was 73.9% (standard error [SE] = 3.6) and 87.2% (SE = 2.7). Relapses occurred early, within 36 months from diagnosis in 28 of 31 (90%) infants. Treatment-related mortality was 3.6%. Age <6 months was a favourable prognostic factor with a 6-year disease-free survival (DFS) of 91% (SE = 9.0) compared with 71.7% (SE = 4.2) in infants >6 months of age (P = 0.04). Patients with high EOI MRD ≥5 × 10-4 had a worse outcome (6-year DFS 61.4% [SE = 12.4], n = 16), compared with patients with undetectable EOI MRD (6-year DFS 87.9% [SE = 6.6], n = 28) or intermediate EOI MRD <5 × 10-4 (6-year DFS 76.4% [SE = 11.3], n = 24; P = 0.02). CONCLUSION: We conclude that young age at diagnosis and low EOI MRD seem favourable prognostic factors in infants with KMT2A-germline ALL and should be considered for risk stratification in future clinical trials.
- MeSH
- akutní lymfatická leukemie komplikace mortalita patologie MeSH
- analýza přežití MeSH
- kojenec MeSH
- lidé MeSH
- prognóza MeSH
- reziduální nádor etiologie patologie MeSH
- výsledek terapie MeSH
- zárodečné buňky MeSH
- Check Tag
- kojenec MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
- MeSH
- akutní lymfatická leukemie genetika patologie MeSH
- delece genu * MeSH
- dítě MeSH
- filadelfský chromozom * MeSH
- jednonukleotidový polymorfismus genetika MeSH
- kohortové studie MeSH
- lidé MeSH
- mutace genetika MeSH
- nádorové biomarkery genetika MeSH
- prognóza MeSH
- sekvenční analýza DNA MeSH
- transkripční faktor Ikaros genetika MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- Publikační typ
- dopisy MeSH
- multicentrická studie MeSH
- práce podpořená grantem MeSH
