Please use this identifier to cite or link to this item: https://hdl.handle.net/2440/116225
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Type: Journal article
Title: Immature MEF2C-dysregulated T-cell leukemia patients have an early T-cell precursor acute lymphoblastic leukemia gene signature and typically have non-rearranged T-cell receptors
Author: Zuurbier, L.
Gutierrez, A.
Mullighan, C.G.
Cante-Barrett, K.
Gevaert, A.O.
de Rooi, J.
Li, Y.
Smits, W.K.
Buijs-Gladdines, J.G.
Sonneveld, E.
Look, A.T.
Horstmann, M.
Pieters, R.
Meijerink, J.P.
Citation: Haematologica: the hematology journal, 2014; 99(1):94-102
Publisher: Ferrata Storti Foundation
Issue Date: 2014
ISSN: 0390-6078
1592-8721
Statement of
Responsibility: 
Linda Zuurbier, Alejandro Gutierrez, Charles G. Mullighan, Kirsten Canté-Barrett, A. Olivier Gevaert, Johan de Rooi, Yunlei Li, Willem K. Smits, Jessica G.C.A.M. Buijs-Gladdines, Edwin Sonneveld, A. Thomas Look, Martin Horstmann, Rob Pieters, and Jules P.P. Meijerink
Abstract: Three distinct immature T-cell acute lymphoblastic leukemia entities have been described including cases that express an early T-cell precursor immunophenotype or expression profile, immature MEF2C-dysregulated T-cell acute lymphoblastic leukemia cluster cases based on gene expression analysis (immature cluster) and cases that retain non-rearranged TRG@ loci. Early T-cell precursor acute lymphoblastic leukemia cases exclusively overlap with immature cluster samples based on the expression of early T-cell precursor acute lymphoblastic leukemia signature genes, indicating that both are featuring a single disease entity. Patients lacking TRG@ rearrangements represent only 40% of immature cluster cases, but no further evidence was found to suggest that cases with absence of bi-allelic TRG@ deletions reflect a distinct and even more immature disease entity. Immature cluster/early T-cell precursor acute lymphoblastic leukemia cases are strongly enriched for genes expressed in hematopoietic stem cells as well as genes expressed in normal early thymocyte progenitor or double negative-2A T-cell subsets. Identification of early T-cell precursor acute lymphoblastic leukemia cases solely by defined immunophenotypic criteria strongly underestimates the number of cases that have a corresponding gene signature. However, early T-cell precursor acute lymphoblastic leukemia samples correlate best with a CD1 negative, CD4 and CD8 double negative immunophenotype with expression of CD34 and/or myeloid markers CD13 or CD33. Unlike various other studies, immature cluster/early T-cell precursor acute lymphoblastic leukemia patients treated on the COALL-97 protocol did not have an overall inferior outcome, and demonstrated equal sensitivity levels to most conventional therapeutic drugs compared to other pediatric T-cell acute lymphoblastic leukemia patients.
Keywords: Precursor T-Cell Lymphoblastic Leukemia-Lymphoma
Rights: ©2013 Ferrata Storti Foundation. This is an open-access paper.
DOI: 10.3324/haematol.2013.090233
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