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https://hdl.handle.net/2440/116225
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dc.contributor.author | Zuurbier, L. | - |
dc.contributor.author | Gutierrez, A. | - |
dc.contributor.author | Mullighan, C.G. | - |
dc.contributor.author | Cante-Barrett, K. | - |
dc.contributor.author | Gevaert, A.O. | - |
dc.contributor.author | de Rooi, J. | - |
dc.contributor.author | Li, Y. | - |
dc.contributor.author | Smits, W.K. | - |
dc.contributor.author | Buijs-Gladdines, J.G. | - |
dc.contributor.author | Sonneveld, E. | - |
dc.contributor.author | Look, A.T. | - |
dc.contributor.author | Horstmann, M. | - |
dc.contributor.author | Pieters, R. | - |
dc.contributor.author | Meijerink, J.P. | - |
dc.date.issued | 2014 | - |
dc.identifier.citation | Haematologica: the hematology journal, 2014; 99(1):94-102 | - |
dc.identifier.issn | 0390-6078 | - |
dc.identifier.issn | 1592-8721 | - |
dc.identifier.uri | http://hdl.handle.net/2440/116225 | - |
dc.description.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. | - |
dc.description.statementofresponsibility | 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 | - |
dc.language.iso | en | - |
dc.publisher | Ferrata Storti Foundation | - |
dc.rights | ©2013 Ferrata Storti Foundation. This is an open-access paper. | - |
dc.source.uri | http://dx.doi.org/10.3324/haematol.2013.090233 | - |
dc.subject | Precursor T-Cell Lymphoblastic Leukemia-Lymphoma | - |
dc.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 | - |
dc.type | Journal article | - |
dc.identifier.doi | 10.3324/haematol.2013.090233 | - |
pubs.publication-status | Published | - |
dc.identifier.orcid | Mullighan, C.G. [0000-0002-1871-1850] | - |
Appears in Collections: | Aurora harvest 3 Medicine publications |
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