摘要
目的探讨异基因造血干细胞移植（allo-HSCT）后EB病毒相关淋巴组织增生性疾病（PTLD）的临床特点、诊治和预后。方法回顾分析262例allo-HSCT的临床资料，移植后9例发生EB病毒相关PTLD，发生率3.44%（9/262）。该9例受者中，男性6例，女性3例，中位年龄19岁；原发病为重型再生障碍性贫血（SAA）6例，急性髓系白血病2例，慢性髓系白血病1例。结果EB病毒相关PTLD的中位发生时间为移植后58 d（44～271 d），其临床表现多为抗感染治疗无效的反复发热，扁桃体和浅表淋巴结肿大。9例受者中，6例为病理诊断，3例为临床诊断；浅表淋巴结受累8例，中枢神经系统（CNS）受累4例，肺部受累2例，骨骼受累1例。发病时，外周血EB病毒DNA中位拷贝数为7.21×10^4拷贝/ml （6.37×10^3～4.56×105拷贝/ml）。4例CNS受累的受者发病时，仅1例受者外周血EB病毒DNA阳性。9例受者治疗后，4例全效，4例部分有效，1例无效。随访28个月（2～48个月），6例受者死亡，3例存活。结论原发病为SAA的受者移植后较白血病受者更易发生EB病毒相关PTLD；减量或停用免疫抑制剂、利妥昔单抗和小剂量供者淋巴细胞输注（DLI）是治疗EB病毒相关PTLD的有效手段。
Objective To summarize the clinical characteristics, diagnosis, treatment and prognosis of EBV related post-transplantation lymphoproliferative diseases （PTLD） after allogeneic hematopoietic stem cell transplantation （allo-HSCT）.MethodsThe clinical data of 262 cases of allo-HSCT were retrospectively, and EBV-associated PTLD occurred in 9 cases after transplantation with a incidence of 3.44% （9/262）. Of the 9 patients, 6 were males and 3 were females, with a median age of 19 years; the primary disease was severe aplastic anemia （SAA） in 6 cases, acute myeloid leukemia in 2 cases and chronic myeloid leukemia in 1 case.ResultsThe occurring median time of EBV associated PTLDs was 58 d （44-271 d）. The clinical manifestations of most PTLD recipients were recurrent fever with no reaction to any antibiotics, antiadoncus and lymphadenectasis. Of the 9 recipients, 6 cases obtained pathological diagnosis, and 3 cases clinical diagnosis. Superficial lymph node and central nervous system （CNS） involved in 8 and 4 recipients, respectively; lung and bone involvement occurred in 2 recipients and 1 case, respectively. The median number of peripheral blood EBV DNA in 9 recipients was 7.21×10^4 copies/ml （6.37×10^3-4.56×105 copies/ml） at the time of onset. EBV DNA in peripheral blood was positive in only one case of 4 CNS recipients. Among 9 recipients after therapy, 4 cases were cured and 4 cases were partially effective, and 1 recipient was ineffective. After follow-up for 28 months （2-48 months）, 6 cases died, and 3 survived.ConclusionIncidence of EBV related PTLD in SAA patients undergoing allo-HSCT is relatively higher than leukemia recipients. Reduction or withdrawal of immunosuppressant, Rituximab and low dose of DLI is effective treatment.
作者
周健
祖璎玲
梁利杰
韩利杰
张莉
符粤文
李珍
林全德
赵慧芳
李玉富
刘艳艳
宋永平
Zhou Jian;Zu Yingling;Liang Lijie;Han Lijie;Zhang Yanli;Fu Yuewen;Li Zhen;Lin Quande;Zhao Hui fang;Li Yufu;Liu Yanyan;Song Yongping.(Department of Hematology, Affiliated Cancer Hospital of Zhengzhou University, Henan Tumor Hospital, Institute of Hematology Henan Province, Zhengzhou 450003, Chin)
出处
《中华器官移植杂志》
CAS
CSCD
北大核心
2018年第2期87-91,共5页
Chinese Journal of Organ Transplantation
关键词
造血干细胞移植
EB病毒
淋巴细胞增殖性疾病
Hematopoietic stem cell transplantation
Epstein-Barr virus
Lymphoproliferative diseases
