Blood, 1 December 2000, Vol. 96, No. 12, pp. 3997-3999
BRIEF REPORT
Successful stem cell transplantation following orthotopic liver
transplantation from the same haploidentical family donor in a girl
with hemophagocytic lymphohistiocytosis
Susanne Matthes-Martin,
Christina Peters,
Alfred Königsrainer,
Gerhard Fritsch,
Thomas Lion,
Andreas Heitger,
Klaus Kapelari,
Martina Kronberger,
Felix Offner,
Fritz Wrba,
Raimund Margreiter, and
Helmut Gadner
From St Anna Children's Hospital, the
Children's Cancer Research Institute, and the
Institute for Clinical Pathology, Vienna, Austria, and the
Department of Transplant Surgery, University Hospital
Innsbruck, the University Children's Hospital, and the
Institute for Pathology, Innsbruck, Austria.
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.
