Abstract
Early T-Cell precursor acute lymphoblastic leukemia (ETP-ALL) is a relatively newly identified subset of T-lineage ALL. There are conflicting results regarding prognosis, and the genetic basis of this condition is variable. Here, we summarize the current status of the field and discuss the role of mutations in the Polycomb Repressive Complex 2 frequently identified in ETP-ALL patients.
Highlights
Specialty section: This article was submitted to Pediatric Hematology and Hematological Malignancies, a section of the journal Frontiers in Pediatrics
In 2009, Coustan-Smith and colleagues reported a subtype of T-lineage acute lymphoblastic leukemia (T-ALL) with transcriptional and surface marker profile similarities to early T-cell precursors (ETP-ALL) [1]
The Meijerink-group identified an immature subgroup of T-ALL with high MEF2C expression, which based on expression profiling clustered separately from other T-ALL cases and was enriched for Early T-Cell precursor acute lymphoblastic leukemia (ETP-ALL) cases [9]
Summary
Specialty section: This article was submitted to Pediatric Hematology and Hematological Malignancies, a section of the journal Frontiers in Pediatrics. Poor outcomes were reported for adult patients with immune-phenotypic ETPALL or T-ALL with a gene expression profile characteristic of ETP-ALL [5,6,7]. The Meijerink-group identified an immature subgroup of T-ALL with high MEF2C expression, which based on expression profiling clustered separately from other T-ALL cases and was enriched for ETP-ALL cases [9].
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