Successful stem cell transplantation following orthotopic liver transplantation from the same haploidentical family donor in a girl with hemophagocytic lymphohistiocytosis.
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)
Matthes-Martin
S
S
St Anna Children's Hospital, the Children's Cancer Research Institute, Vienna, Austria.
Peters
C
C
Königsrainer
A
A
Fritsch
G
G
Lion
T
T
Heitger
A
A
Kapelari
K
K
Kronberger
M
M
Offner
F
F
Wrba
F
F
Margreiter
R
R
Gadner
H
H
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