Haploidentical hematopoietic stem cell transplantation for adult patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis

被引:11
作者
Li, Zhihui [1 ]
Wang, Yini [1 ]
Wang, Jingshi [1 ]
Zhang, Jia [1 ]
Wang, Zhao [1 ]
机构
[1] Capital Med Univ, Beijing Friendship Hosp, Dept Hematol, 95 Yong An Rd, Beijing 100050, Peoples R China
基金
北京市自然科学基金;
关键词
Hemophagocytic lymphohistiocytosis; adult; Epstein-Barr virus; haploidentical; allogeneic hematopoietic stem cell transplantation; BONE-MARROW-TRANSPLANTATION; LYMPHOPROLIFERATIVE DISEASE; SALVAGE THERAPY; JAPAN;
D O I
10.1080/10428194.2017.1330467
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
To assess the efficacy of haploidentical hematopoietic stem cell transplantation (HSCT) in adult patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH), we retrospectively analyzed 30 cases that presented at our institution. At the time of HSCT, 20 patients (66.7%) had achieved a response after receiving HLH-94 or salvage therapies. All patients underwent myeloablative conditioning followed by peripheral blood HSCT from their related, haploidentical donors. Twenty-six patients (86.7%) achieved donor cell engraftment. Of these, 23 (88.5%) achieved complete chimerism and three (11.5%) demonstrated mixed chimerism. Reactivated EBV infection was found in 25 (96.2%). Acute graft-versus-host disease occurred in 18 (69.2%), with grade I-II in 11 patients and grade III-IV in seven. Chronic graft-versus-host disease occurred in six (23.1%). Nineteen patients survived until the end of follow-up. The threeyear overall survival rate was 63.3%. Our results indicate that haploidentical HSCT is an effective treatment for adult patients with EBV-HLH.
引用
收藏
页码:77 / 84
页数:8
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