Heidi Kristine Støve1, Julie Damgaard Sandahl1, Jonas Abrahamsson2, Peter H Asdahl1, Erik Forestier3, Shau-Yin Ha4, Kirsi Jahnukainen5, Ólafur G Jónsson6, Birgitte Lausen7, Josefine Palle8, Bernward Zeller9, Henrik Hasle1. 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, Umeå University Hospital, Umeå, 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
BACKGROUND: The prognostic significance of extramedullary leukemia (EML) in childhood acute myeloid leukemia is not clarified. PROCEDURE: This population-based study included 315 children from the NOPHO-AML 2004 trial. 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. 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.
BACKGROUND: The prognostic significance of extramedullary leukemia (EML) in childhood acute myeloid leukemia is not clarified. PROCEDURE: This population-based study included 315 children from the NOPHO-AML 2004 trial. 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. 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.
Authors: Anilkumar Gopalakrishnapillai; Colin E Correnti; Kristina Pilat; Ida Lin; Man Kid Chan; Ashok D Bandaranayake; Christopher Mehlin; Anne Kisielewski; Darcy Hamill; Allison J Kaeding; Soheil Meshinchi; James M Olson; Edward Anders Kolb; Sonali P Barwe Journal: Cancers (Basel) Date: 2021-11-26 Impact factor: 6.639
Authors: Allison J Kaeding; Sonali P Barwe; Anilkumar Gopalakrishnapillai; Rhonda E Ries; Todd A Alonzo; Robert B Gerbing; Colin Correnti; Michael R Loken; Lisa Eidenschink Broderson; Laura Pardo; Quy H Le; Thao Tang; Amanda R Leonti; Jenny L Smith; Cassie K Chou; Min Xu; Tim Triche; Steven M Kornblau; E Anders Kolb; Katherine Tarlock; Soheil Meshinchi Journal: Blood Adv Date: 2021-05-11