Introduction
Genetic alterations of acute lymphoblastic leukemia (ALL) involved in
various signaling pathways are associated with the disease
pathogenesis.Moreover,they may play a role as predictive biomarkers for
selecting the treatment protocol and adjusting risk
stratification.Inactivation of the tumor suppressor gene CDKN2Ain the
9p21.3 locus,which has been implicated in many cancer,can occurby
deletion,methylation, or
mutation[2].CDKN2A
gene deletion is commonly present in pediatric ALL which can be detected
by fluorescent in-situ
hybridization(FISH)[3].It showed that CDKN2A
deletion occurred in 21% BCP-ALL and 50% T-ALL
patients[4].Under normal conditions,the CDKN2A
gene encodes two proteins,p16INK4a and
p14ARF.These two proteins specifically inactivate
cyclin/CDK4/6 complexes that block cell division during the G1/S phase
of the cell cycle[5].A lot of research found that
the deletion of CDNK2A is not only associated with T-lineage ALL but
also B-lineage ALL[4,6-7].
Despite the high frequency of CDKN2A deletion in pediatric ALL,the
prognosis importance in pediatric ALL is still inconclusive.Most of the
results thought the deletion of CDKN2A was associated with pediatric ALL
recurrence[8-9].While Mirebeau D reported that
inactivation of CDKN2A gene did not influence B-lineage acute
lymphoblastic leukemia of childhood’ outcome in
2006[6].
In this article we tried to describe the clinical-biological
characteristics and prognostic factors in pediatric ALL patients with
CDKN2A deletion.
Methods