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儿童异基因造血干细胞移植相关性血栓性微血管病9例病例分析并文献复习 被引量:1

Allogeneic hematopoietic stem cell transplantation - associated thrombotic microangiopathy: 9 cases report of children and literature review
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摘要 目的总结分析儿童异基因造血干细胞移植(allo-HSCT)相关血栓性微血管病(TA-TMA)的临床特征、疗效及转归。方法回顾性分析2008年1月至2017年11月于首都医科大学附属北京儿童医院血液肿瘤中心接受allo-HSCT后发生TA-TMA的9例患儿临床资料、治疗及预后特点。结果纳入研究的283例行allo-HSCT患儿,9例(3.2%)发生TA-TMA。9例患儿中,男5例,女4例,中位年龄94个月(39-129个月),中位发病时间为移植后63 d(6-342 d),血小板中位数为44×10^9/L[(7-75)×10^9/L],血红蛋白中位数为76 g/L(40-105 g/L),乳酸脱氢酶(LDH)中位水平为594 U/L(445-1 386 U/L)。所有患儿中5例出现神经系统症状,4例出现肾脏受累,6例出现胃肠道受累表现。TA-TMA确诊后的主要治疗措施为减停钙调磷酸酶体抑制剂,血浆置换,利妥昔单抗及去纤苷。4例经治疗后病情得到控制,治疗有效,5例患儿治疗无效死亡。结论TA-TMA是allo-HSCT后的致死性并发症,可导致全身多器官、系统功能损害,累及〉2个系统提示预后不良,联合治疗效果优于单药治疗,选择个体化的治疗方案对减缓TA-TMA病情进展及改善预后至关重要。 ObjectiveTo analyze the clinical characteristics, treatment and prognosis of allogeneic hemato-poietic stem cell transplantation(allo-HSCT)-associated thrombotic microangiopathy (TA-TMA) in children.MethodsThe clinical information, treatment and prognosis of 9 cases with TA-TMA hospitalized following allo-HSCT from January 2008 to November 2017 in Hematology Oncology Center, Beijing Children′s Hospital, Capital Medical University were retrospectively analyzed.ResultsOf all the 283 allo-HSCT recipients, 9 patients (3.2%) were diagnosed as TA-TMA.Among them, there were 5 male and 4 female, with a median age of 94 months (39-129 months). The median time to of diagnosis was 63 days (6-342 days) after HSCT.Additionally, the median platelet counts, hemoglobin and lactate dehydrogenase(LDH) levels were 44×10^9/L[(7-75)×10^9/L], 76 g/L(40-105 g/L) and 594 U/L(445-1 386 U/L). Neurological symptoms were found in 5 of the patients, 4 had kidney involvement, and 6 had gastrointestinal involvement.The major treatment of TA-TMA was plasma exchange, Rituximab and defibrotide instead of the use of calcineurin inhibitors.Finally, 4 patients achieved response after treatment, 5 children died of ineffective treatment.ConclusionTA-TMA is a fatal complication after allo-HSCT.It can lead to multi-organ and multi-systems dysfunction.If there are more than 2 systems involved in TA-TMA, it suggests poor prognosis.The combined therapy is better than monotherapy, and the selective individual treatment of TA-TMA is essential.
作者 贺艺璇 He Yixuan;Li Sidan;Wu Runhui;Wang Bin;Zhu Guanghua;Yang Jun;Wang Kai;Yan Yan;Jia Chenguang;Qin Maoquan(National Children's Medical Center,Beijing Key Laboratory of Pediatric Hematology Oncolog;National Key Discipline of Pediatrics,Ministry of Educatio;Ministry of Education(MOE)Key Laboratory of Major Diseases in Childre;Hematology Oncology Center,Beijing Children's Hospital,Capital Medical University,Beifing 100045,China)
出处 《中华实用儿科临床杂志》 CSCD 北大核心 2018年第18期1419-1422,共4页 Chinese Journal of Applied Clinical Pediatrics
关键词 移植相关血栓性微血管病 造血干细胞移植 儿童 治疗 Transplantation-associated thrombotic microangiopathy Hematopoietic stem cell transplantation Child Treatment
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