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7.21×104拷贝/ml (6.37×103～4.56×105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.

abstractsObjective 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).Methods The 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 Results The 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 × 104 copies/ml (6.37 × 103-4.56 × 105 copies/ml) at the time of onset.EBV DNA in peripheral blood was positive in only one ease 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.Conclusion Incidence 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.