X linked lymphoproliferative disease in a United Kingdom family
a St Mary's Hospital,
Manchester: Department of Child Health, b Immunogenetics Laboratory, c Royal
Manchester Children's Hospital, Manchester:
Department of Paediatric Oncology/Haematology
Correspondence to: Dr G M Taylor, Immunogenetics Laboratory, St Mary's Hospital, Hathersage Road, Manchester M13 0JH, UK. email: gmtaylor{at}man.ac.uk
Accepted 15 December
1997
X linked lymphoproliferative disease (XLP; Duncan's disease)
is a rare disorder affecting boys and characterised by a defective immune response to Epstein- Barr virus caused by a mutation in a gene
located at chromosome Xq25. Three siblings with XLP in a single UK
family are reported and the variation in phenotypic expression of the
disease in these siblings described. One of the siblings with life
threatening fulminant infectious mononucleosis was successfully treated
by chemotherapy, followed by bone marrow transplantation using an
unaffected brother as the donor. A healthy baby boy recently born into
the family was identified as carrying the defective maternal X
chromosome using molecular genetic linkage analysis. This family
illustrates the extent of present understanding of this often fatal condition.
© 1998 by Archives of Disease in Childhood
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