Extramedullary leukemia in children with acute myeloid leukemia: A population‐based cohort study from the Nordic Society of Pediatric Hematology and Oncology (NOPHO) |
| |
| Authors: | Heidi Kristine Støve Julie Damgaard Sandahl Jonas Abrahamsson Peter H. Asdahl Erik Forestier Shau‐Yin Ha Kirsi Jahnukainen Ólafur G. Jónsson Birgitte Lausen Josefine Palle Bernward Zeller Henrik Hasle |
| |
| Affiliation: | 1. Department of Pediatrics, Aarhus University Hospital, Aarhus, Denmark;2. Department of Pediatrics, Institution for Clinical Sciences, Queen Silvia Children's Hospital, Gothenburg, Sweden;3. Department of Medical Biosciences and Genetics, Ume? University Hospital, Ume?, Sweden;4. Department of Pediatrics, Hong Kong Pediatric Hematology & Oncology Study Group, Queen Mary Hospital, Hong Kong, China;5. Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland;6. Department of Pediatrics, Landspitalinn, Reykjavik, Iceland;7. Department of Pediatrics and Adolescent Medicine, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark;8. Department of Woman's and Children's Health, Uppsala University, Uppsala, Sweden;9. Department of Pediatric Medicine, Oslo University Hospital, Oslo, Norway |
| |
| Abstract: | 1 Background The prognostic significance of extramedullary leukemia (EML) in childhood acute myeloid leukemia is not clarified. 2 Procedure This population‐based study included 315 children from the NOPHO‐AML 2004 trial. 3 Results At diagnosis, 73 (23%) patients had EML: 39 (12%) had myeloid sarcoma, 22 (7%) had central nervous system disease, and 12 (4%) had both. EML was associated with young age (median age: 2.6 years), a high white blood cell count (median: 40 × 109/l), M5 morphology (40%), and 11q23/MLL (KMT2A) rearrangements (34%). No patient received involved field radiotherapy. Five‐year event‐free survival did not differ significantly between the EML and the non‐EML patients (54% vs. 45%, P = 0.57), whereas 5‐year overall survival (OS) was significantly lower in the EML group (64% vs. 73%, P = 0.04). The risk of induction death was significantly higher for EML patients (8% vs. 1%, P = 0.002). There was a trend toward a lower risk of relapse for EML patients (5‐year cumulative incidence of relapse 33% vs. 49%, P = 0.16). Traumatic lumbar puncture did not adversely affect survival in this cohort. 4 Conclusions EML was associated with increased risk of induction death impacting the OS. No patients relapsed at the primary site of the myeloid sarcoma despite management without radiotherapy. |
| |
| Keywords: | acute myeloid leukemia central nervous system disease children chloroma extramedullary leukemia |
|
|
