Paediatric XXXX with the t(7;12)(q36;p13) rearrangement: a review of the biological and clinical management aspects.

@entity1 @entity2008 @entity1778 @entity3052
UNASSIGNED: The presence of @entity2008 is one of the most important criteria for @entity1778 diagnosis and management. @entity1 @entity1778 is a rare disease that affects @entity1 in their first year of life. It has been estimated that approximately one third of @entity1 with @entity3052 harbour the t(7;12)(q36;p13) rearrangement in their leukaemic blasts. However, the WHO classification of @entity3052 does not yet include the t(7;12) as a separate entity among the different genetic subtypes, although the presence of this @entity2008 has been associated with an extremely poor clinical outcome. Currently, there is no consensus treatment for t(7;12) @entity1778 @entity1 . However, with the inferior outcome with the standard induction therapy, stem cell transplantation may offer a better chance for disease control. A better insight into the chromosome biology of this entity might shed some light into the pathogenic mechanisms arising from this chromosomal translocation, that at present are not fully understood. Further work is needed to improve our understanding of the molecular and genetic basis of this disorder. This will hopefully open some grounds for possible tailored treatment for this subset of very young @entity1 with inferior disease outcome. This review aims at highlighting the cytogenetic features that characterise the @entity1778 for a better detection of the abnormality in the diagnostic setting. We also review treatment and clinical outcome in the cases reported to date.
