Allogeneic hematopoietic stem cell transplantation for hemophagocytic syndrome.

Ruijuan SUN; Juan XIAO; Jianping ZHANG; Yue LU; Zhijie WEI; Huili ZHU; Yuan SUN

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Allogeneic hematopoietic stem cell transplantation for hemophagocytic syndrome.

Author: Ruijuan SUN ¹ ; Juan XIAO ¹ ; Jianping ZHANG ¹ ; Yue LU ¹ ; Zhijie WEI ¹ ; Huili ZHU ¹ ; Yuan SUN ²
Author Information

1. Department of Pediatric Hematology, Beijing Daopei Hospital, Beijing 100192, China.
2. Department of Pediatric Hematology, Beijing Daopei Hospital, Beijing 100192, China. Email: sunyuan20021@sina.com.
Publication Type:Journal Article
MeSH: Adolescent; Child; Child, Preschool; Cyclosporine; administration & dosage; Cytomegalovirus Infections; epidemiology; prevention & control; Female; Graft vs Host Disease; epidemiology; prevention & control; Hematopoietic Stem Cell Transplantation; adverse effects; Humans; Immunosuppressive Agents; administration & dosage; Lymphohistiocytosis, Hemophagocytic; mortality; therapy; Male; Survival Rate; Tissue Donors; Transplantation Conditioning; methods; Treatment Outcome
From: Chinese Journal of Pediatrics 2014;52(2):94-98
CountryChina
Language:Chinese
Abstract:
OBJECTIVETo observe the conditioning regimen, efficacy and side effects of allogeneic hematopoietic stem cell transplantation (allo-HSCT) for hemophagocytic lymphohistiocytosis (HLH).
METHODFrom 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.
RESULTThree 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.
CONCLUSIONThe allo-HSCT was successful in treating primary and refractory hemophagocytic syndrome.