title: Successful stem cell transplantation following orthotopic liver transplantation from the same haploidentical family donor in a girl with hemophagocytic lymphohistiocytosis.
abstract: The case of a 4-month-old girl with familial hemophagocytic lymphohistiocytosis is described. The patient underwent stem cell transplantation from her haploidentical mother 2 months after receiving a living-related liver transplant from the same donor for acute hepatic failure. Conditioning regimen consisted of 16 mg/kg busulfan, 200 mg/kg cyclophosphamide, 10 mg/kg thiothepa, and antithymocyte globulin. Myeloid engraftment occurred on day +10, but CD3(+) cells of recipient origin remained. To convert the T-cell chimerism, the patient received donor lymphocyte infusion on day +43, and subsequently the allelic pattern changed to complete donor genotype on day +57. Four months after stem cell transplantation the patient is disease free, with complete donor chimerism in bone marrow and stable hepatic graft function without any immunosuppressive therapy. (Blood. 2000;96:3997-3999)
authors.affiliation: St Anna Children's Hospital, the Children's Cancer Research Institute, Vienna, Austria.
authors.forename: S
authors.initials: S
authors.lastname: Matthes-Martin
authors.forename: C
authors.initials: C
authors.lastname: Peters
authors.forename: A
authors.initials: A
authors.lastname: Königsrainer
authors.forename: G
authors.initials: G
authors.lastname: Fritsch
authors.forename: T
authors.initials: T
authors.lastname: Lion
authors.forename: A
authors.initials: A
authors.lastname: Heitger
authors.forename: K
authors.initials: K
authors.lastname: Kapelari
authors.forename: M
authors.initials: M
authors.lastname: Kronberger
authors.forename: F
authors.initials: F
authors.lastname: Offner
authors.forename: F
authors.initials: F
authors.lastname: Wrba
authors.forename: R
authors.initials: R
authors.lastname: Margreiter
authors.forename: H
authors.initials: H
authors.lastname: Gadner
mesh_terms: D001521:Behavior Therapy; D005260:Female; D006084:Graft Rejection / Q000517:prevention & control; D006085:Graft Survival / Q000276:immunology; D006239:Haplotypes / Q000276:immunology; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods / Q000592:standards; D015616:Histiocytosis, Non-Langerhans-Cell / Q000150:complications / Q000601:surgery*; D006801:Humans; D007108:Immune Tolerance; D016130:Immunophenotyping; D007165:Immunosuppression Therapy / Q000379:methods; D007223:Infant; D017114:Liver Failure, Acute / Q000209:etiology / Q000601:surgery; D016031:Liver Transplantation / Q000276:immunology / Q000379:methods* / Q000592:standards; D016176:T-Lymphocyte Subsets; D014019:Tissue Donors; D018183:Transplantation Chimera