重型地中海贫血造血干细胞移植28天后血细胞减少原因分析  

Analysis of cytopenia beyond day 28 after allogeneic hematopoietic stem cell transplantation in childhood thalassemia major

作　　者：周小辉[1] 廖建云[1] 吴学东[1] 刘秋君[1] 陈佳奇[1] 冯晓勤[1] 何岳林[1] 裴夫瑜[1] 刘华颖[1] 石磊[1] 李春富[1] 

机构地区：[1]南方医科大学南方医院儿科,广州510515

出　　处：《中国小儿血液与肿瘤杂志》2013年第4期164-168,共5页Journal of China Pediatric Blood and Cancer

摘　　要：目的探讨重型β地中海贫血(TM)患儿行造血干细胞移植(HSCT)治疗28天后血细胞减少(CB-28PT)的原因,为治疗提供临床经验。方法对2009年1月-2012年11月在本中心行HSCT治疗的187例TM患儿进行单因素、多因素logistic回归模型评估与CB-28PT相关的风险因素。结果 187例行HSCT治疗的TM患儿中,39例发生CB-28PT,34例经治疗后血细胞恢复正常。单因素回归分析HLA配型(P=0.003)、供者年龄(P=0.01)是CB-28PT发生的危险因素,HLA相合同胞供者组(MSD)与变更供者组(AD)相比,MSD组不易发生CB-28PT(P=0.028)。多因素分析仅HLA配型是CB-28PT发生的危险因素(P=0.023)。混合效应模型分析显示白细胞计数和CsA血药浓度(P<0.001)、甲基强的松龙剂量(P<0.001)之间存在正相关。结论 HLA配型不相合是CB-28PT发生的最主要原因。我们认为TM患儿CB-28PT可能是慢性移植物抗宿主病的一种表现。Objective To explore the causes of cytopenia beyond day 28 （CB-28PT） after allergenic hematopoietic stem cell transplantation （HSCT） in children with thalassemia major （TM）,and to provide a guideline for the post-transplant treatment.Methods A total of 187 TM patients undergone the HSCT from Jan 2009 to Oct 2012 were analyzed retrospectively.CB-28PT is defined as the white blood cell （WBC） counts less than 3.0 × 103/mn3 for 4 weeks or longer without B19 virus,EBV and CMV infections.Results Thirty-nine cases were identified as CB-28PT,and 34 cases recovered to the normal level of WBC after treatment.The HLA mismatch （P =0.003） and donor age （P =0.01） were showed as the risk factors for CB-28PT by using univariate analysis.Compared to the alternative donor transplant,HLA-matched sibling donor transplantation （MST） had a protective effect on CB-28PT （P =0.028）.The HLA mismatch was analyzed as the only risk factor for CB-28PT by using multivariate analysis （P =0.023）.There were significant differences among WBC counts,CsA blood concentration （P ＜0.001） and methylprednisolone dose （P ＜ 0.001）.Conclusions HLA mismatch is the major risk factor of CB-28PT,which may be a manifestation of chronic graft-versus-host disease.

关 键 词：地中海贫血 造血干细胞移植 血细胞减少 HLA配型不相合 慢性移植物抗宿主病 

分 类 号：R556[医药卫生—血液循环系统疾病]