Outcomes of allogeneic haematopoietic cell transplantation for myelofibrosis in children and adolescents: the retrospective study of the EBMT Paediatric Diseases WP.
Jacek WachowiakJacques-Emmanuel GalimardArnaud DalissierRawad RihaniHawazen Al-SaediRobert F WynnJean-Hugues DalleRégis Peffault de LatourPetr SedlacekAdriana BalduzziThomas SchroederIvana BodovaMarta Gonzalez VicentBernd GruhnRose-Marie HamladjiGergely KrivanKatharine PatrickAgnieszka Sobkowiak-SobierajskaPolina StepenskyAli UnalPersis Jal AmroliaPérez-Martínez AntonioFanny RiallandMahmoud AljurfAntonella IsgroAmos TorenMarc B BieringsSelim CorbaciogluKrzyzstof KalwakPublished in: Bone marrow transplantation (2024)
This retrospective study evaluated 35 children (median age 5.2 years; range 0.4-18) with myelofibrosis (MF), including 33 with primary myelofibrosis and 2 with secondary myelofibrosis transplanted from matched sibling donor (MSD) (n = 17) or non-MSD (n = 18) between 2000 and 2022. Conditioning was usually chemotherapy-based (n = 33) and myeloablative (n = 32). Fifteen patients received bone marrow (BM), 14 haematopoietic cells (HC) from peripheral blood (PB), and 6 from cord blood (CB). Day +100 acute GvHD II-IV incidence was significantly lower after MSD-haematopoietic cell transplantation (MSD-HCT) than after non-MSD-HCT [18.8% (4.3-41.1) vs 58.8% (31-78.6); p = 0.01]. Six-year non-relapse mortality (NRM) was 18% (7.1-32.8), relapse incidence was 15.9% (5.6-30.9), progression-free survival (PFS) was 66.1% (47-79.7), GvHD-free relapse-free survival was 50% (30.6-66.7), and overall survival (OS) was 71.1% (51.4-84). Six-year PFS and OS were significantly higher after BM transplantation compared to HCT from other sources [85.1% (52.3-96.1) vs 50.8% (26.3-71), p = 0.03, and 90.9% (50.8-98.7) vs 54% (28.1-74.2), p = 0.01, respectively], whereas NRM was significantly lower [0% vs 32% (12.3-53.9); p = 0.02]. This first multicentre study on outcomes of allogeneic HCT in children with myelofibrosis proves feasibility and curative effect of transplantation in these children, suggests that bone marrow transplantation is associated with better outcomes, and indicates the need for further studies.
Keyphrases
- free survival
- bone marrow
- cell therapy
- cell cycle arrest
- stem cell transplantation
- cord blood
- young adults
- peripheral blood
- mesenchymal stem cells
- risk factors
- single cell
- allogeneic hematopoietic stem cell transplantation
- cell death
- end stage renal disease
- newly diagnosed
- stem cells
- ejection fraction
- intensive care unit
- induced apoptosis
- prognostic factors
- emergency department
- type diabetes
- liver failure
- squamous cell carcinoma
- cardiovascular events
- cardiovascular disease
- metabolic syndrome
- locally advanced
- acute myeloid leukemia
- radiation therapy
- adipose tissue
- mechanical ventilation
- patient reported outcomes
- endoplasmic reticulum stress