Transplantation for adrenoleukodystrophy with HLA-A and B nonidentical paternal marrow: report of one case.
We report the result of allogeneic bone marrow transplantation (BMT) in a 14-year-old boy who was neurologically severely involved with the childhood form of adrenoleukodystrophy (ALD) and received marrow from his HLA-A and B nonidentical, MLC-nonreactive paternal donor without T-cell depletion processing. Bone marrow transplantation corrected the excess content of very long chain fatty acid in plasma but did not arrest the deterioration of the neurological status during 3.5-year post-transplant follow-up period. Since partially matched or unrelated donors have been applied to clinical BMT successfully with current new techniques, ALD patients will have a better prognosis when they are transplanted in status of mild and early involvement. Our first experience may be helpful in more trials of BMT for genetic leukodystrophy in Taiwan.
Department of Pediatrics, En Chu Kong Hospital, San-Shia, Taipei, Taiwan, R.O.C.
H C
HC
Lin
K H
KH
Lin
P J
PJ
Wang
D000293:Adolescent; D000326:Adrenoleukodystrophy / Q000097:blood / Q000276:immunology / Q000601:surgery*; D016026:Bone Marrow Transplantation / Q000276:immunology*; D015234:HLA-A Antigens / Q000032:analysis*; D015235:HLA-B Antigens / Q000032:analysis*; D006650:Histocompatibility Testing; D006801:Humans; D019520:Living Donors; D008297:Male
D015234:HLA-A Antigens; D015235:HLA-B Antigens