Highlights
CRLF2
overexpression associates with
IKZF1
deletions that lead to a dominant-negative effect and with
IKZF1
plus.
Paediatric patients with a high load expression of IK4 isoform presented higher
CRLF2
transcript levels.
CRLF2
overexpression and
IKZF1
deletions conferred poorer prognosis both to paediatric patients treated with RELLA05 protocol as well as to adult patients.
BackgroundNear-tetraploid (model #81-103) and near-triploid (model #67-81) karyotypes are found in around 1% of childhood acute lymphoblastic leukemia. Due to its rarity, these two cytogenetic subgroups are generally included in the hyperdiploid group (model # > 51). Therefore separate informations about these two subgroups are limited to a few reports. Some studies found that near-tetraploidy is relatively more frequent in higher median ages and it is associated to Frech-American-British Classification subtype L2. Although the mechanisms by which leukemic blast cells divide is still unclear, studies have suggested that hyperdiploidy, near-triploidy and near-tetraploidy do not seem to share the same mechanism.FindingsHerewith, we present a new childhood T-acute lymphoblastic leukemia case of near-tetraploid karyotype with loss of two p53-gene copies, characterized in detail by cytogenetic and molecular studies.ConclusionWe suggest that p53 is a good target gene to be screened, once p53 is one of the main effectors of cell cycle checkpoints.
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