title: [Allogeneic hematopoietic stem cell transplantation for hemophagocytic syndrome].
abstract: OBJECTIVE
  To observe the conditioning regimen, efficacy and side effects of allogeneic hematopoietic stem cell transplantation (allo-HSCT) for hemophagocytic lymphohistiocytosis (HLH).
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  The allo-HSCT was successful in treating primary and refractory hemophagocytic syndrome.
authors.affiliation: Department of Pediatric Hematology, Beijing Daopei Hospital, Beijing 100192, China.
authors.forename: Ruijuan
authors.initials: R
authors.lastname: Sun
authors.affiliation: Department of Pediatric Hematology, Beijing Daopei Hospital, Beijing 100192, China.
authors.forename: Juan
authors.initials: J
authors.lastname: Xiao
authors.affiliation: Department of Pediatric Hematology, Beijing Daopei Hospital, Beijing 100192, China.
authors.forename: Jianping
authors.initials: J
authors.lastname: Zhang
authors.affiliation: Department of Pediatric Hematology, Beijing Daopei Hospital, Beijing 100192, China.
authors.forename: Yue
authors.initials: Y
authors.lastname: Lu
authors.affiliation: Department of Pediatric Hematology, Beijing Daopei Hospital, Beijing 100192, China.
authors.forename: Zhijie
authors.initials: Z
authors.lastname: Wei
authors.affiliation: Department of Pediatric Hematology, Beijing Daopei Hospital, Beijing 100192, China.
authors.forename: Huili
authors.initials: H
authors.lastname: Zhu
authors.affiliation: Department of Pediatric Hematology, Beijing Daopei Hospital, Beijing 100192, China. Email: sunyuan20021@sina.com.
authors.forename: Yuan
authors.initials: Y
authors.lastname: Sun
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D016572:Cyclosporine / Q000008:administration & dosage; D003586:Cytomegalovirus Infections / Q000453:epidemiology / Q000517:prevention & control; D005260:Female; D006086:Graft vs Host Disease / Q000453:epidemiology / Q000517:prevention & control*; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000401:mortality / Q000628:therapy*; D008297:Male; D015996:Survival Rate; D014019:Tissue Donors; D019172:Transplantation Conditioning / Q000379:methods; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents; D016572:Cyclosporine