Leukemia  in  Children with X-
SCID
l Similar insertional mutagenesis events in both
children
l Unregulated C cytokine receptor gene inserted
into LMO2 locus
l Activation of LMO2, a proliferative gene
l A rare event in an early T cell/HSC that leads to a
leukemic transformation
l Slow growth and eventual proliferation of the
clone
l May be prevented by regulated C cytokine
receptor gene