儿童异基因造血干细胞移植后并发免疫性溶血性贫血10例：单中心研究

发布时间：2018-11-04 17:04

【摘要】：目的:总结儿童异基因HSCT后AIHA的发生率、发病机制、危险相关因素、治疗效果,以期为临床提供参考。方法:回顾性分析2007年6月至2015年12月31日在重庆医科大学附属儿童医院血液肿瘤中心进行异基因HSCT后并发AIHA的患者的移植特征及临床特点,总结并探讨AIHA的发生率;单因素分析移植相关因素(包括:疾病类型、供者类型、干细胞来源、预处理方案、GVHD预防方案、HLA相合性、ABO血型相合性、供受者性别相合性、急性GVHD分度及是否并发慢性GVHD等)与AIHA的关系;并进一步分析AIHA的独立危险因素,探讨AIHA的临床治疗效果及其与总体生存率的关系。单因素分析采用卡方检验,组间对比采用log-rank检验,多因素分析采用Cox比例风险回归模。结果:本研究中97例行异基因HSCT的患者中10例发生AIHA,总体发生率为10.3%;1年累计发病率为5.6%;18.2%WAS患者移植后发生AIHA(8/44);41.2%合并慢性GVHD的患儿在移植后发生不同程度AIHA(7/17)。AIHA的中位发生时间为D+93天;WAS患者组移植后AIHA的累积发生率显著高于血液系统恶性疾病患者组(P=0.043);供受者ABO血型次侧不合移植组患者移植后AIHA的累积发生率高于血型相合移植组患者(P=0.044);而供受者血型主侧不合组患者移植后早期AIHA的累积发生率与血型相合移植组患者或次侧不合移植组患者相比,差异无统计学意义。10例患者AIHA的发生均在免疫功能完全重建之前;除2例患者在造血重建前即发生AIHA外,其余8例患者AIHA的发生均在造血基本重建以后;合并慢性GVHD是移植后并发AIHA的独立危险因素;30%移植后AIHA为难治性AIHA,利妥昔单抗对难治性AIHA可能有效。结论:儿童异基因HSCT后AIHA的发生率相对较高,尤其是WAS患者;无关供者移植后AIHA发生率较MSD患者高,合并慢性GVHD是并发AIHA的独立危险因素;AIHA的发生可能与HSCT后受者体内的免疫失调程度相关;利妥昔单抗可能对难治性AIHA有效。目前仍需要大样本研究对AIHA的发生率、危险因素、发生机制及有效治疗措施进行更全面的分析。
[Abstract]:Objective: to summarize the incidence, pathogenesis, risk factors and therapeutic effect of AIHA after allogeneic HSCT in children. Methods: the transplant characteristics and clinical characteristics of patients with AIHA after allogeneic HSCT were analyzed retrospectively from June 2007 to December 31 2015 in the Children's Hospital affiliated to Chongqing Medical University. The incidence of AIHA was summarized and discussed. Univariate analysis of transplant related factors (including disease type, donor type, stem cell source, preconditioning protocol, GVHD prophylaxis, HLA compatibility, ABO blood type compatibility, donor gender compatibility, The relationship between acute GVHD grading and chronic GVHD, etc.) and AIHA; Furthermore, the independent risk factors of AIHA were analyzed, and the clinical effect of AIHA and its relationship with overall survival rate were discussed. Chi-square test was used for single factor analysis, log-rank test was used for inter-group contrast, and Cox proportional risk regression model was used for multivariate analysis. Results: in this study, the overall incidence of AIHA, was 10.3 in 97 patients with allogeneic HSCT, the cumulative incidence in one year was 5.6 / 18.2was and the incidence of AIHA was 8 / 44 (8 / 44). 41.2% of the children with chronic GVHD had different degrees of AIHA after transplantation (the median time of 7 / 17). AIHA was D 93 days), the cumulative incidence of AIHA in the WAS group was significantly higher than that in the hematological malignancy group (P0.043). The cumulative incidence of AIHA in the donor group with ABO blood group subtransplantation was higher than that in the matched donor group (P0. 044). The cumulative incidence of early AIHA in donor blood group was higher than that in blood matching group or subtransplantation group. There was no significant difference. The occurrence of AIHA in 10 patients was before the complete reconstruction of immune function. With the exception of 2 patients who developed AIHA before hematopoietic reconstitution, 8 patients with AIHA occurred after basic hematopoietic reconstitution, and chronic GVHD was the independent risk factor of AIHA after transplantation. After transplantation, 30% of the patients with AIHA were resistant to AIHA, and Rituximab might be effective in the treatment of refractory AIHA. Conclusion: the incidence of AIHA after allogeneic HSCT is higher in children, especially in WAS patients, the incidence of AIHA in unrelated donors is higher than that in MSD patients, and chronic GVHD is an independent risk factor for AIHA. The occurrence of AIHA may be related to the degree of immune disorder in recipients after HSCT, and Rituximab may be effective for refractory AIHA. There is still a need for a more comprehensive analysis of the incidence, risk factors, pathogenesis and effective treatment of AIHA.
【学位授予单位】：重庆医科大学
【学位级别】：硕士
【学位授予年份】：2016
【分类号】：R725.5

【相似文献】