Allogeneic hematopoietic stem cell transplantation for hemophagocytic syndrome / 中华儿科杂志
Chinese Journal of Pediatrics
; (12): 94-98, 2014.
Article
in Zh
| WPRIM
| ID: wpr-288781
Responsible library:
WPRO
ABSTRACT
<p><b>OBJECTIVE</b>To observe the conditioning regimen, efficacy and side effects of allogeneic hematopoietic stem cell transplantation (allo-HSCT) for hemophagocytic lymphohistiocytosis (HLH).</p><p><b>METHOD</b>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.</p><p><b>RESULT</b>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.</p><p><b>CONCLUSION</b>The allo-HSCT was successful in treating primary and refractory hemophagocytic syndrome.</p>
Full text:
1
Index:
WPRIM
Main subject:
Therapeutics
/
Tissue Donors
/
Epidemiology
/
Survival Rate
/
Mortality
/
Treatment Outcome
/
Cyclosporine
/
Cytomegalovirus Infections
/
Hematopoietic Stem Cell Transplantation
/
Transplantation Conditioning
Type of study:
Prognostic_studies
Limits:
Adolescent
/
Child
/
Child, preschool
/
Female
/
Humans
/
Male
Language:
Zh
Journal:
Chinese Journal of Pediatrics
Year:
2014
Type:
Article