左卡尼汀在地中海贫血患儿骨髓移植治疗过程中的作用  

Application of Levocarnitine in the treatment of thalassemia during bone marrow transplantation

作　　者：刘素丽[1] 李春富[1] 邹纬[2] 何岳林[1] 吴学东[1] 冯晓勤[1] 李娜[1] 裴夫瑜[1] 石磊[1] 

机构地区：[1]南方医科大学附属南方医院,广州510515 [2]广州军区广州总医院

出　　处：《山东医药》2010年第11期31-33,共3页Shandong Medical Journal

摘　　要：目的探讨左卡尼汀在儿童重型β-地中海贫血异基因造血干细胞移植(allo-HSCT)过程中的应用价值。方法将41例拟行allo-HSCT的重型地贫患儿随机分为观察组25例及对照组16例,两组均采用环磷酰胺+白舒非+氟达拉宾+抗胸腺细胞球蛋白为主的预处理化疗方案,静脉予环孢素A(CsA)及口服吗替麦考酚酯(MMC)预防移植物抗宿主病(GVHD)。观察组移植前5 d(-5 d)至移植后20 d(+20 d)静脉应用左卡尼汀1 g,2次/d。监测两组-5 d^+31 d内血浆脑利钠肽前体(pro-BNP)水平及临床心功能不全相关指标。结果两组治疗后BNP均升高,+5^+15 d达峰值,+25 d后逐渐下降至正常范围;+6 d开始观察组pro-BNP显著低于对照组(P<0.05);观察组心功能不全事件发生率显著低于对照组(P=0.044)。结论左卡尼汀可有效降低重型地贫患儿在allo-HSCT过程中的心功能不全事件发生率,其机制可能通过为心肌提供足够能量及减少有害物质生成从而降低心肌损害程度。Objective To investigate the value of Levoearnitine in the treatment of pediatrics with β-thalassemia major during allogene hematopoietic stem cell transplantation （allo-HSCT）. Methods Forty-one pediatric patients with β- thalassemia major underwent planned allo-HSCT were divided into observed group（ n = 25）and control group（ n = 16 ）, both groups were given conditioning regimes included Busulfex ＋ Cyelophosphamide ＋ antithymocyte globulin ＋ Fludarabine, HSCT was followed by Cielosporin A（CsA） or Myeophenolate Mofetil Capsules（MMC） to prevent graft versus host disease （GVHD）. Patients in observed group were given levoearnitine 1 gram, twice a day via intravenous injection from 5 days （ -5 d） before HSCT to 20 days after（ ＋ 20 d）. The plasma pro-BNP and clinical cardiac insufficiency related index were monitored from -5 d to ＋ 31 d. Results BNP values of both groups increased after HSCT, achieved the peak at 5 to 15 days after HSCT, and dropped to normal level at about 25 days after HSCT. The pro-BNP value of the observed group was significantly lower than that of the control group early as 6 days after HSCT（ P 〈 0.05 ）. Incidence of cardiac event in the observed group was significantly lower than that in the control group（ P = 0. 044）. Conclusions Levoearnitine is probably an effective drug to reduce occurrence rate of cardiac event during HSCT for pediatric patients with β-thalassemia major. The mechanism is maybe that Levoearnitine can offer enough energy to cardiac muscle and decrease harmful substants to lessen heart injury.

关 键 词：左卡尼汀 心功能不全 重型Β-地中海贫血 异基因造血干细胞移植 

分 类 号：R556[医药卫生—血液循环系统疾病]