Early T-Cell Precursor ALL and Beyond: Immature and Ambiguous Lineage T-ALL Subsets

A wide range of immature acute leukemias (AL), ranging from acute myeloid leukemias with minimal differentiation to acute leukemias with an ambiguous lineage, i.e., acute undifferentiated leukemias and mixed phenotype acute leukemia with T- or B-plus myeloid markers, cannot be definitely assigned to...

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Main Authors: Eulàlia Genescà, Roberta la Starza
Format: Article
Language:English
Published: MDPI AG 2022-04-01
Series:Cancers
Subjects:
Online Access:https://www.mdpi.com/2072-6694/14/8/1873
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author Eulàlia Genescà
Roberta la Starza
author_facet Eulàlia Genescà
Roberta la Starza
author_sort Eulàlia Genescà
collection DOAJ
description A wide range of immature acute leukemias (AL), ranging from acute myeloid leukemias with minimal differentiation to acute leukemias with an ambiguous lineage, i.e., acute undifferentiated leukemias and mixed phenotype acute leukemia with T- or B-plus myeloid markers, cannot be definitely assigned to a single cell lineage. This somewhat “grey zone” of AL expresses partly overlapping features with the most immature forms of T-cell acute lymphoblastic leukemia (T-ALL), i.e., early T-cell precursor ALL (ETP-ALL), near-ETP-ALL, and pro-T ALL. These are troublesome cases in terms of precise diagnosis because of their similarities and overlapping phenotypic features. Moreover, it has become evident that they share several genomic alterations, raising the question of how their phenotypes reflect distinct AL entities. The aim of this review was to provide a systematic overview of the genetic events associated with immature T-ALL and outline their relationship with treatment choices and outcomes, especially looking at the most recent preclinical and clinical studies. We wish to offer a basis for using the genetic information for new diagnostic algorithms, in order to better stratify patients and improve their management with more efficient and personalized therapeutic options. Understanding the genetic profile of this high-risk T-ALL subset is a prerequisite for changing the current clinical scenario.
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spelling doaj.art-74f7ec7190354f169281ec23d136068c2023-12-01T01:04:29ZengMDPI AGCancers2072-66942022-04-01148187310.3390/cancers14081873Early T-Cell Precursor ALL and Beyond: Immature and Ambiguous Lineage T-ALL SubsetsEulàlia Genescà0Roberta la Starza1Institut d’Investigació Contra la Leucemia Josep Carreras (IJC), Campus ICO-Germans Trias i Pujol, Universitat Autònoma de Barcelona, 08916 Badalona, SpainHematology and Immunology Section, Department of Medicine and Surgery, CREO, Università degli Studi di Perugia, 06132 Perugia, ItalyA wide range of immature acute leukemias (AL), ranging from acute myeloid leukemias with minimal differentiation to acute leukemias with an ambiguous lineage, i.e., acute undifferentiated leukemias and mixed phenotype acute leukemia with T- or B-plus myeloid markers, cannot be definitely assigned to a single cell lineage. This somewhat “grey zone” of AL expresses partly overlapping features with the most immature forms of T-cell acute lymphoblastic leukemia (T-ALL), i.e., early T-cell precursor ALL (ETP-ALL), near-ETP-ALL, and pro-T ALL. These are troublesome cases in terms of precise diagnosis because of their similarities and overlapping phenotypic features. Moreover, it has become evident that they share several genomic alterations, raising the question of how their phenotypes reflect distinct AL entities. The aim of this review was to provide a systematic overview of the genetic events associated with immature T-ALL and outline their relationship with treatment choices and outcomes, especially looking at the most recent preclinical and clinical studies. We wish to offer a basis for using the genetic information for new diagnostic algorithms, in order to better stratify patients and improve their management with more efficient and personalized therapeutic options. Understanding the genetic profile of this high-risk T-ALL subset is a prerequisite for changing the current clinical scenario.https://www.mdpi.com/2072-6694/14/8/1873immature T-ALLETP-ALLdiagnosisgenomicsoutcometreatment
spellingShingle Eulàlia Genescà
Roberta la Starza
Early T-Cell Precursor ALL and Beyond: Immature and Ambiguous Lineage T-ALL Subsets
Cancers
immature T-ALL
ETP-ALL
diagnosis
genomics
outcome
treatment
title Early T-Cell Precursor ALL and Beyond: Immature and Ambiguous Lineage T-ALL Subsets
title_full Early T-Cell Precursor ALL and Beyond: Immature and Ambiguous Lineage T-ALL Subsets
title_fullStr Early T-Cell Precursor ALL and Beyond: Immature and Ambiguous Lineage T-ALL Subsets
title_full_unstemmed Early T-Cell Precursor ALL and Beyond: Immature and Ambiguous Lineage T-ALL Subsets
title_short Early T-Cell Precursor ALL and Beyond: Immature and Ambiguous Lineage T-ALL Subsets
title_sort early t cell precursor all and beyond immature and ambiguous lineage t all subsets
topic immature T-ALL
ETP-ALL
diagnosis
genomics
outcome
treatment
url https://www.mdpi.com/2072-6694/14/8/1873
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