Multifocal remitting-relapsing cerebral demyelination twenty years following allogeneic bone marrow transplantation.
We report a case study of a female who received an allogeneic bone marrow transplantation (BMT) from a sex-mismatched related donor and who, after a twenty-year interval, developed an acute fulminant biopsy-proven demyelinating disorder of cerebral white matter which followed a remitting-relapsing chronic course. In situ hybridization studies using Y-chromosome-specific markers revealed Y-chromosome-positive mononuclear cells in biopsy samples of white matter. Magnetic resonance imaging (MRI) studies of the asymptomatic healthy male donor showed multiple white matter lesions. These observations suggest that donor lymphocytes were sensitized to central nervous system (CNS) antigens prior to or at the time of transplantation but remained dormant for 20 years before becoming activated to cause widespread demyelination.
Kelly
P
P
Richmond Institute of Neurology and Neurosurgery, Beaumont Hospital, Dublin, Ireland.
Staunton
H
H
Lawler
M
M
Brennan
P
P
Jennings
S
S
Unger
E R
ER
Sung
J H
JH
Farrell
M A
MA
D000328:Adult; D001345:Autoradiography; D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D002540:Cerebral Cortex / Q000503:physiopathology*; D004247:DNA / Q000032:analysis; D003711:Demyelinating Diseases / Q000175:diagnosis / Q000209:etiology*; D005260:Female; D006239:Haplotypes; D006801:Humans; D017403:In Situ Hybridization; D008279:Magnetic Resonance Imaging; D008297:Male; D009443:Neuritis / Q000209:etiology; D016511:Severe Combined Immunodeficiency / Q000628:therapy; D012737:Sex Factors; D013997:Time Factors; D018183:Transplantation Chimera; D014184:Transplantation, Homologous / Q000009:adverse effects; D014998:Y Chromosome
D004247:DNA