嵌合状态对造血干细胞移植后贫血儿童结局的影响。
Influence of Mixed Chimerism on Outcome in Children With Anaemia After Haematopoietic Stem Cell Transplantation.
机构信息
Department of Pediatric Hematology, Oncology and Transplantology, Medical University of Lublin, Laboratory of Genetic Diagnostics, Lublin, Poland
Department of Pediatric Hematology, Oncology and Transplantology, Medical University of Lublin, Lublin, Poland.
出版信息
In Vivo. 2019 Nov-Dec;33(6):2051-2057. doi: 10.21873/invivo.11703.
BACKGROUND/AIM: In patients with non-malignant diseases, mixed chimerism is not a rare phenomenon. The clinical impacts of chimerism following allogeneic haematopoietic stem cell transplantation (allo-HSCT) in children with congenital anaemia (CA) and severe aplastic anaemia (SAA) were analysed.
PATIENTS AND METHODS
We studied twenty-seven consecutive children with congenital and acquired anaemia who had undergone allogeneic haematopoietic stem cell transplantations. In the observed group of patients, the median of the follow-up was 6.12 years (2.00-14.8 years).
RESULTS
Overall survival (OS) did not depend on the type of disease p=0.1. OS did not significantly differ in patients who received more than 5x10/kg stem cells (91%) and those who received less than 5x10/kg (85%) (p=0.61). Two patterns of stable mixed chimerism (SMC) were observed: SMC (95-97% cells of the donor), and SMC with a fluctuation between 50-90% of the cells of the donor. None of the surviving patients received immunosuppression treatments of chronic Graft-versus-Host Disease (cGvHD).
CONCLUSION
Our results showed that mixed chimerism did not influence the survival of children with congenital and aplastic anaemia following allo-HSCT.
背景/目的：在非恶性疾病患者中，混合嵌合体并非罕见现象。本研究旨在分析儿童先天性贫血（CA）和重型再生障碍性贫血（SAA）患者接受异基因造血干细胞移植（allo-HSCT）后嵌合体的临床影响。
方法
我们研究了 27 例连续接受 allo-HSCT 的先天性和获得性贫血儿童患者。在观察性患者组中，中位随访时间为 6.12 年（2.00-14.8 年）。
结果
总生存（OS）与疾病类型无关（p=0.1）。接受超过 5x10/kg 干细胞的患者（91%）与接受低于 5x10/kg 干细胞的患者（85%）OS 无显著差异（p=0.61）。观察到两种稳定混合嵌合体（SMC）模式：SMC（供者细胞的 95-97%）和 SMC 波动在供者细胞的 50-90%之间。无存活患者接受慢性移植物抗宿主病（cGvHD）的免疫抑制治疗。
结论
本研究结果表明，混合嵌合体不影响 allo-HSCT 后先天性和再生障碍性贫血儿童的生存。
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