非血缘脐带血移植成功治疗复发难治性EB病毒相关噬血细胞综合征合并肠穿孔1例并文献复习  

Case report of unrelated cord blood transplantation for the treatment of recurrent refractory Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis with intestinal perforation and literature review

作　　者：路娜丹 王玮琳 汤苗苗 丁艳杰 苏淑芳[1] 李白[1] 王叨[1] Lu Nadan;Wang Weilin;Tang Miaomiao;Ding Yanjie;Su Shufang;Li Bai;Wang Dao(Department of Pediatrics,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]郑州大学第一附属医院儿科,郑州450052

出　　处：《中华实用儿科临床杂志》2022年第24期1899-1902,共4页Chinese Journal of Applied Clinical Pediatrics

摘　　要：目的探讨非血缘脐带血移植(UCBT)治疗儿童复发难治性EB病毒相关噬血细胞综合征(EBV-HLH)的临床经验及疗效。方法回顾性分析2015年9月郑州大学第一附属医院儿科诊治的1例复发难治性EBV-HLH合并肠穿孔,最终接受UCBT治愈患儿的临床资料,并进行文献复习。结果患儿,男,1岁6个月,因"发热15 d,皮疹9 d"为主诉入院,主要表现为高热,肝、脾、淋巴结大,快速进展的全血细胞减少、肝功能损害,骨髓涂片可见吞噬血细胞,2015年9月确诊为EBV-HLH,按国际组织细胞协会制定的HLH-2004方案化疗,维持期间2次复发,给予挽救性二线方案"培门冬酰胺酶、阿霉素脂质体、依托泊苷、甲泼尼龙"(L-DEP方案)化疗,化疗后评估噬血细胞综合征指标完全缓解,突发肠穿孔,紧急外科手术行小肠造瘘术,病情稳定后,给予"氟达拉滨+白消安+环磷酰胺"方案(Flu+BU+CY方案)预处理后行UCBT,全程静脉营养支持,移植后第13天中性粒细胞植入,第35天血小板植入,嵌合率为100%,植入成功;移植后第15天出现肝小静脉闭塞征,移植后第22天出现真菌性肺炎,移植后第26天出现皮肤移植物抗宿主病(GVHD)Ⅱ度,给予相应治疗好转;移植后第49天行二期肠造瘘关瘘术;现随访至移植后70个月,患儿一般状况良好,病情持续缓解,无慢性GVHD及其他合并症。结论异基因造血干细胞移植可能是治疗儿童复发难治性EBV-HLH的唯一有效手段;无合适同胞或非血缘供者时,非血缘脐带血干细胞可作为移植物来源;肠穿孔术后肠造瘘不是移植禁忌。Objective To examine the clinical experience and efficacy of unrelated cord blood transplantation(UCBT)in the treatment of recurrent refractory Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis(EBV-HLH)in children.Methods The clinical data of a patient with recurrent refractory EBV-HLH and intestinal perforation who was treated by UCBT in Department of Pediatrics,the First Affiliated Hospital of Zhengzhou University in September 2015 and finally cured were retrospectively analyzed.Meanwhile,literature was reviewed.Results The patient,male,1 year and 6 months,was admitted to the hospital with"fever for 15 days,rash for 9 days"as the main complaint,mainly manifested as high fever,large liver,spleen,lymph nodes,rapidly progressing pancytopenia,liver function damage,phagocytic blood cells on bone marrow smear,diagnosed as EBV-HLH in September 2015.The patient received chemotherapy according to the HLH-2004 protocol developed by the International Association of Cell Societies.During the treatment,he suffered two recurrence during the maintenance period,and a second-line rescue treatment was adopted,namely,"Pegaspargase,Doxorubicin liposome,Etoposide and Methylprednisolone"(L-DEP regimen)chemotherapy.The complete relief of diagnostic indexes for hemophagocytic lymphohistiocytosis was evaluated after chemotherapy.The patient developed sudden intestinal perforation and underwent emergency surgical surgery,enteroenterostomy.After the condition was stabilized,the patient was pretreated with the"Fludarabine+Busulfan+Cyclophosphamide"(Flu+BU+CY)therapy and then treated with UCBT,with intravenous nutritional support provided during the entire process.Neutrophil and platelet implantation was implemented on day 13 and day 35 after transplantation,respectively.The chimeric rate was 100%,and the implantation was a success.Hepatic veno-occlusive disease,fungal pneumonia and skin graft-versus-host disease(GVHD)Ⅱoccurred on the 15th day,22nd day and 26th day after transplantation,respectively.The corresponding sympt

关 键 词：脐带血 EB病毒感染 噬血细胞综合征 肠穿孔 

分 类 号：R457.7[医药卫生—治疗学] R725.5[医药卫生—临床医学]