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Bone marrow transplantation for late infantile metachromatic leukodystrophy: Pathogenic investigation for graft rejection
Author(s) -
NAGAI TOSHIRO,
KANEKO TAKASHI,
SHICHIJOU HIROMI,
KARATO TAKAKO,
MARUYAMA AKIKO,
TSUCHIYA YUTAKA
Publication year - 1993
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/j.1442-200x.1993.tb03081.x
Subject(s) - metachromatic leukodystrophy , medicine , bone marrow transplantation , leukodystrophy , transplantation , pathology , bone marrow , surgery , disease
A Japanese boy aged 2 years 11 months with late infantile metachromatic leukodystrophy underwent bone marrow transplantation (BMT) from his human leukocyte antigen (HLA) identical but mixed lymphocyte culture reactive father. Chimerism and increased arylsulfatase A activities of leukocytes had been observed with retarded progression of neurological deterioration during the first 3 months post‐BMT. Graft rejection gradually occurred and donor cells were almost completely eliminated from the patient at 1 year after BMT. The process of neurodegeneration progressed clinically and neuroradiologically. Three possible reasons for the pathogenesis of graft rejection are: (i) T cell depletion of donor marrow cells as graft‐versus‐host disease (GVHD) prophylaxis; (ii) a slightly weak conditioning regimen; and (iii) a small number of marrow cells transplanted. It is stressed that as BMT is still a preliminary therapy for metachromatic leukodystrophy indications, conditioning, and GVHD prophylaxis for BMT should be considered individually.