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Acute lymphoblastic leukemias with deletion of 11q23 or a novel inversion
(11)(p13q23) lack MLL gene rearrangements and have favorable clinical
features
SC Raimondi, JL Frestedt, CH Pui, JR Downing, DR Head, JH Kersey and FG Behm
Department of Pathology, St Jude Children's Research Hospital, Memphis, TN
38105, USA.
Balanced translocations affecting the 11q23 region are among the most
frequent chromosomal abnormalities in childhood acute lymphoblastic
leukemia (ALL), comprising 5% to 6%. These cases consistently have a
rearranged MLL gene and are associated with high-risk presenting features,
hyperleukocytosis and younger age, and a poor treatment outcome. To assess
the clinical and biologic significance of 11q23- associated structural
chromosomal abnormalities other than translocations, we studied 17 cases of
childhood ALL [14 with del(11)(q23) and 3 with inv(11)(p12q23)] that were
identified among 785 cases with successful chromosome analysis. In contrast
to reported cases with 11q23 and MLL gene rearrangement, our series was
characterized by relatively low leukocyte counts (median, 15.1 x 10(9)/L),
expression of CD10 antigen but not myeloid-associated CD15 and CDw65
antigens, a relatively high frequency of T-cell immunophenotypes, and a
generally favorable prognosis. All 13 cases with interpretable molecular
analysis lacked MLL gene rearrangements. We suggest that most cases with
deletions or inversions affecting the 11q23 region represent clinically and
biologically different entities as compared with those defined by 11q23
translocation.
Volume 86,
Issue 5,
pp. 1881-1886,
09/01/1995
Copyright © 1995 by The American Society of Hematology
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