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目的:分析重型β地中海贫血患者同胞脐血移植后的造血和免疫重建情况,评价同胞脐血移植治疗重型β地中海贫血效果。方法:2例患者移植后均为稳定骨髓植入,分别于移植后1,3,6,9 个月检测患者外周血T淋巴细胞亚群(CD3+、CD3+CD4+、CD3+CD8+)、B淋巴细胞(CD19+ )和NK细胞(CD56+ )数量,动态分析脐血移植后患者的免疫重建。结果:2例患者移植后中性粒细胞绝对计数≥0.5×109/L的时间分别为第21天和第12天,血小板计数≥50.0×109/L的时间分别为第53天和第39天,血常规恢复正常时间分别为第71 天和第59天。T细胞亚群在6个月内均恢复正常;B细胞和NK细胞在3个月内均恢复正常。CD4+/CD8+ 比值恢复正常的时间分别为3个月和9个月。B细胞和NK细胞的恢复比T细胞快。2例患者均发生急性Ⅰ度移植物抗宿主病。结论:同胞脐血移植后患者免疫重建快且稳定,急性移植物抗宿主病程度轻。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 hematopoietic and immune reconstructions after sibling cord blood transplantation in patients with β-thalassemia major and to evaluate the effect of cord blood transplantation in patients with severe β-thalassemia. Methods: All the 2 patients received stable bone marrow transplantation after transplantation. The levels of T lymphocyte subsets (CD3 +, CD3 + CD4 +, CD3 + CD8 +) and B lymphocytes in peripheral blood were measured at 1, 3, 6 and 9 months after transplantation The number of cells (CD19 +) and NK cells (CD56 +) were dynamically analyzed for immune reconstitution in patients after cord blood transplantation. Results: The absolute neutrophil count ≥0.5 × 109 / L in 2 patients after transplantation was 21 and 12 days respectively, and the platelet counts ≥50.0 × 109 / L were on days 53 and 39 respectively , Blood routine recovery time was 71 days and 59 days. T cell subsets returned to normal within 6 months; B cells and NK cells returned to normal within 3 months. CD4 + / CD8 + ratio returned to normal for 3 months and 9 months respectively. B cells and NK cells recover faster than T cells. Both patients developed acute first degree graft-versus-host disease. Conclusion: The immune reconstitution in patients with cord blood transplantation is fast and stable, and the severity of acute graft-versus-host disease is light. Sib umbilical cord blood transplantation is an effective method to cure severe β-thalassemia.