A successful transplantation of sibling marrow in a patient with the X- linked hyper-IgM syndrome is reported. Engraftment of HLA-identical marrow cells was obtained, although complicated by grade I acute graft-versus-host disease. Expression of the CD40 ligand (CD40L, CD154) by activated T-cells from the recipient remained at low levels until 10 months after the transplantation, but then normalized. The patient is now fully competent in immune function without any episodes of severe infection 24 months later. Conclusion Allogeneic bone marrow transplantation is a reasonable therapeutic option for X-linked hyper-IgM syndrome if HLA-matched family donors are available. Whether dysregulation of CD40L expression causes post-transplant immunological abnormalities remains to be clarified.
- Bone marrow transplantation
- CD40 ligand (CD154)
- X-linked hyper-IgM syndrome
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health