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目的分析儿童重型β-地中海贫血同胞脐血移植后的免疫重建情况,评价同胞脐血移植治疗重型β-地中海贫血的效果。方法11例患者移植后均为稳定骨髓植入,分别于移植后1、3、6、12、18~24、36~48个月检测患者外周血T淋巴细胞亚群(CD3+细胞、CD3+CD4+细胞、CD3+CD8+细胞)、B淋巴细胞(CD19+细胞)和自然杀伤细胞(CD3-CD56+NK细胞)数量,动态分析脐血移植后患者的免疫重建。结果 14例患者脐血移植后11例获得植入,植入率为78.6%。11例患者移植后中性粒细胞绝对计数≥0.5×109/L、血小板计数≥20.0×109/L和≥50.0×109/L的中位时间分别为17、48、53 d。总淋巴细胞和T淋巴细胞亚群在12个月内均恢复正常;B细胞和NK细胞分别在6个月和3个月内恢复正常。B细胞和NK细胞的恢复比T细胞快。11例患者发生急性移植物抗宿主病(GVHD),其中Ⅰ度急性GVHD9例(81.8%),Ⅱ度急性GVHD 2例(18.2%)。结论同胞脐血移植是根治重型β-地中海贫血的有效方法 。
Objective To analyze the immune reconstitution of cord blood transplantation in children with severe β-thalassemia and to evaluate the effect of cord blood transplantation on severe β-thalassemia in children. Methods All 11 patients received stable bone marrow transplantation after transplantation. The levels of T lymphocyte subsets (CD3 + cells, CD3 + CD4 + CD3 + CD8 + cells), B lymphocytes (CD19 + cells) and natural killer cells (CD3-CD56 + NK cells) were measured. The immune reconstitution of patients after cord blood transplantation was dynamically analyzed. Results Of the 14 cases, 11 cases received cord blood transplantation and the implantation rate was 78.6%. The median neutrophil count ≥0.5 × 109 / L, platelet count ≥20.0 × 109 / L and ≥50.0 × 109 / L in 11 patients were respectively 17,48 and 53 days after transplantation. Total lymphocytes and T lymphocyte subsets returned to normal within 12 months; B cells and NK cells returned to normal within 6 months and 3 months respectively. B cells and NK cells recover faster than T cells. Eleven patients developed acute graft-versus-host disease (GVHD), of which 9 were grade I acute GVHD (81.8%) and 2 were acute GVHD (18.2%). Conclusion Stroke cord blood transplantation is an effective method to cure severe β-thalassemia.