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Monosomy 7 myeloproliferative disease in children with neurofibromatosis,
type 1: epidemiology and molecular analysis
KM Shannon, J Watterson, P Johnson, P O'Connell, B Lange, N Shah, P Steinherz, YW Kan and JR Priest
Department of Pediatrics, Naval Hospital, Oakland, CA 94627.
Loss of constitutional heterozygosity is a common molecular feature of
cancers in which inactivation of one or more tumor suppressor genes is
thought to contribute to tumorigenesis. Recent evidence suggests that the
gene responsible for neurofibromatosis, type 1 (NF-1), belongs to this
class of heritable cancer genes. Children with NF-1 show an increased
incidence of myeloid leukemia, including juvenile chronic myelogenous
leukemia (JCML) and, perhaps, the myeloproliferative syndrome (MPS)
associated with bone marrow monosomy 7 (Mo 7). We have investigated five
children with Mo 7: three with NF-1 and two others with suggestive evidence
of NF-1. Southern blotting experiments performed in four patients showed no
loss of heterozygosity in bone marrow specimens using probes linked to the
NF-1 locus on the long arm of chromosome 17. Both of our patients with
familial NF-1 inherited the disease from their mothers, as did 14 of 19
other cases of myeloid leukemia in children with familial NF-1. Seventeen
of these 21 children were boys. Myeloid leukemia developed in 12 boys and
four girls who inherited NF-1 from their mothers, and in five boys who
inherited the disease from their fathers. Father-to-daughter transmission
was not observed. Taken together, the presence of chromosome 7 deletions in
the leukemias of children with NF-1, a pattern of inheritance favoring
maternal transmission of NF-1, and the marked predilection for boys to
develop JCML and Mo 7 suggest a multistep mechanism of oncogenesis in which
epigenetic factors might play a role. Further investigation is required to
determine if the NF-1 genes in the leukemic bone marrows of these patients
have acquired point mutations or small deletions.
Volume 79,
Issue 5,
pp. 1311-1318,
03/01/1992
Copyright © 1992 by The American Society of Hematology
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