Department of Pediatric Oncology, Hematology and Transplantology, University of Medical Sciences, Poznan, Poland.
EBMT Paris Study Office, Paris, France.
Bone Marrow Transplant. 2024 Aug;59(8):1057-1069. doi: 10.1038/s41409-024-02286-3. Epub 2024 Apr 16.
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.
这项回顾性研究评估了 35 名患有骨髓纤维化的儿童(中位年龄 5.2 岁;范围 0.4-18 岁),包括 33 名原发性骨髓纤维化和 2 名继发性骨髓纤维化患者,他们均于 2000 年至 2022 年期间接受了来自匹配的同胞供体(MSD)(n=17)或非 MSD(n=18)的异基因造血干细胞移植(allo-HCT)。预处理通常为基于化疗(n=33)和清髓性(n=32)。15 名患者接受了骨髓(BM)移植,14 名患者接受了外周血(PB)造血细胞(HC)移植,6 名患者接受了脐带血(CB)移植。MSD-HCT 后第 100 天急性移植物抗宿主病(GVHD)Ⅱ-Ⅳ级的发生率明显低于非 MSD-HCT [18.8%(4.3-41.1)vs 58.8%(31-78.6);p=0.01]。6 年非复发死亡率(NRM)为 18%(7.1-32.8),复发率为 15.9%(5.6-30.9),无进展生存期(PFS)为 66.1%(47-79.7),GVHD 无复发生存期为 50%(30.6-66.7),总生存期(OS)为 71.1%(51.4-84)。与其他来源的造血干细胞移植(HCT)相比,BM 移植后 6 年的 PFS 和 OS 明显更高[85.1%(52.3-96.1)vs 50.8%(26.3-71),p=0.03;90.9%(50.8-98.7)vs 54%(28.1-74.2),p=0.01],而 NRM 明显更低[0% vs 32%(12.3-53.9);p=0.02]。这项关于儿童骨髓纤维化患者接受 allo-HCT 后结局的首次多中心研究证明了在这些儿童中进行移植的可行性和疗效,提示骨髓移植与更好的结局相关,并表明需要进一步研究。