TY - JOUR
T1 - Immunological reconstitution by allogeneic bone marrow transplantation in a child with the X-linked hyper-IgM syndrome
AU - Kawai, S.
AU - Sasahara, Y.
AU - Minegishi, M.
AU - Tsuchiya, S.
AU - Fujie, H.
AU - Ohashi, Y.
AU - Kumaki, S.
AU - Konno, T.
N1 - Funding Information:
Acknowledgements We thank the nurses for their enduring care of and devotion to the patient and Ms. E. Nitanai for her technical assistance. This work was in part supported by grants from the Ministry of Education, Science, Sports, and Culture, and the Ministry of Health and Welfare, Japan.
Copyright:
Copyright 2007 Elsevier B.V., All rights reserved.
PY - 1999
Y1 - 1999
N2 - 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.
AB - 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.
KW - Bone marrow transplantation
KW - CD40 ligand (CD154)
KW - X-linked hyper-IgM syndrome
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U2 - 10.1007/s004310051099
DO - 10.1007/s004310051099
M3 - Article
C2 - 10333122
AN - SCOPUS:0032921464
VL - 158
SP - 394
EP - 397
JO - European Journal of Pediatrics
JF - European Journal of Pediatrics
SN - 0340-6199
IS - 5
ER -