Sun Ruijuan, Xiao Juan, Zhang Jianping, Lu Yue, Wei Zhijie, Zhu Huili, Sun Yuan
Department of Pediatric Hematology, Beijing Daopei Hospital, Beijing 100192, China.
Department of Pediatric Hematology, Beijing Daopei Hospital, Beijing 100192, China. Email:
Zhonghua Er Ke Za Zhi. 2014 Feb;52(2):94-8.
To observe the conditioning regimen, efficacy and side effects of allogeneic hematopoietic stem cell transplantation (allo-HSCT) for hemophagocytic lymphohistiocytosis (HLH).
From 2010 to 2012, a total of 11 cases after allo-HSCT were evaluated including 8 cases with familial hemophagocytic lymphohistiocytosis (FHL) and 3 cases with Epstein-Barr virus (EBV) related HLH. Allo-HSCT from HLA haploidentical HSCT was performed for 3 cases and unrelated allo-HSCT for 8 cases; 7 cases underwent allo-HSCT with conditioning regimen of etoposide (VP16), busulphan (Bu), fludarabine (Flu) and antilymphocyte globulin (ATG) and 4 cases with Flu, melphalan (Mel) and ATG. Cyclosporine (CsA) or tacrolimus, mycophenolate (MMF) and methorexate (MTX) were used for prevention of graft versus host disease (GVHD). Four cases received anti-CD25 MoAbs, 7 cases received cord blood and 1 of them received haploidentical bone marrow to prevent GVHD.
Three cases died after allo-HSCT. The median overall survival time of the 8 cases evaluated was 585 days (154-1 115 d). All the patients were successfully engrafted. Acute GVHD (aGVHD) occurred in 8 cases, including 3 cases of gradeI/II and 5 cases of grade III/IV. Chronic GVHD (cGVHD) occurred in 4 cases. Seven cases had cytomegalovirus (CMV) reactivation.
The allo-HSCT was successful in treating primary and refractory hemophagocytic syndrome.
观察异基因造血干细胞移植（allo-HSCT）治疗噬血细胞性淋巴组织细胞增生症（HLH）的预处理方案、疗效及副作用。
2010年至2012年，共评估11例allo-HSCT术后患者，其中8例为家族性噬血细胞性淋巴组织细胞增生症（FHL），3例为EB病毒（EBV）相关HLH。3例行单倍型相合造血干细胞移植（HLA haploidentical HSCT），8例行非血缘allo-HSCT；7例采用依托泊苷（VP16）、白消安（Bu）、氟达拉滨（Flu）及抗淋巴细胞球蛋白（ATG）进行预处理，4例采用Flu、美法仑（Mel）及ATG进行预处理。采用环孢素（CsA）或他克莫司、霉酚酸酯（MMF）及甲氨蝶呤（MTX）预防移植物抗宿主病（GVHD）。4例接受抗CD25单克隆抗体，7例接受脐血，其中1例接受单倍型相合骨髓预防GVHD。
3例allo-HSCT术后死亡。8例评估患者的中位总生存时间为585天（154 - 1115天）。所有患者均成功植入。8例发生急性移植物抗宿主病（aGVHD），其中Ⅰ/Ⅱ级3例，Ⅲ/Ⅳ级5例。4例发生慢性移植物抗宿主病（cGVHD）。7例巨细胞病毒（CMV）重新激活。
allo-HSCT治疗原发性及难治性噬血细胞综合征成功。
