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Mutations of the ras proto-oncogenes in childhood monosomy 7
A Neubauer, K Shannon and E Liu
Division of Hematology and Oncology, University of North Carolina, Chapel
Hill 27599-7295.
ras gene mutations are the most frequent molecular changes found in the
preleukemic syndromes of adults and may play a role in initiating these
diseases and in their progression to acute leukemia. However, little is
known about the incidence or importance of these genetic mutations in
childhood myeloproliferative states (MPS). The bone marrow (BM) monosomy 7
syndrome accounts for a large percentage of childhood MPS. Although the
duration of the MPS is quite variable, children with monosomy 7 eventually
develop acute myeloid leukemia (AML). We investigated 20 children (13 with
MPS, 7 with AML) with BM monosomy 7 or 7q- for the presence of ras gene
mutations using the polymerase chain reaction and hybridization with
mutation-specific oligonucleotides. Mutations of N-ras and K-ras were
detected in three children. Two patients carrying a ras mutation were in
the myeloproliferative phase, and one had acute leukemia. All three
patients with ras mutations either died of their disease or relapsed after
BM transplantation as compared with 8 of 17 without ras mutations. However,
this difference is not statistically significant (P = .14, not
significant). We conclude that ras mutations are observed in childhood
monosomy 7, though less frequently than in adult MDS, and may play a
limited role in the progression of this disease to acute leukemia. More
patients are needed to address the prognostic role of ras mutations in this
rare disease.
Volume 77,
Issue 3,
pp. 594-598,
02/01/1991
Copyright © 1991 by The American Society of Hematology
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