TY  - JOUR
A1  - Neumann, Martin
A1  - Heesch, Sandra
A1  - Gökbuget, Nicola
A1  - Schwartz, Stefan
A1  - Schlee, Cornelia
A1  - Benlasfer, Ouidad
A1  - Farhadi-Sartangi, Nasrin
A1  - Thibaut, Julia
A1  - Burmeister, Thomas
A1  - Hoelzer, Dieter
A1  - Hofmann, Wolf-Karsten
A1  - Thiel, Eckhard
A1  - Baldus, Claudia
T1  - Clinical and molecular characterization of early T-cell precursor leukemia : a high-risk subgroup in adult T-ALL with a high frequency of FLT3 mutations
T2  - Blood cancer journal
N2  - A subgroup of pediatric acute T-lymphoblastic leukemia (T-ALL) was characterized by a gene expression profile comparable to that of early T-cell precursors (ETPs) with a highly unfavorable outcome. We have investigated clinical and molecular characteristics of the ETP-ALL subgroup in adult T-ALL. As ETP-ALL represents a subgroup of early T-ALL we particularly focused on this cohort and identified 178 adult patients enrolled in the German Acute Lymphoblastic Leukemia Multicenter studies (05/93–07/03). Of these, 32% (57/178) were classified as ETP-ALL based on their characteristic immunophenotype. The outcome of adults with ETP-ALL was poor with an overall survival of only 35% at 10 years, comparable to the inferior outcome of early T-ALL with 38%. The molecular characterization of adult ETP-ALL revealed distinct alterations with overexpression of stem cell-related genes (BAALC, IGFBP7, MN1, WT1). Interestingly, we found a low rate of NOTCH1 mutations and no FBXW7 mutations in adult ETP-ALL. In contrast, FLT3 mutations, rare in the overall cohort of T-ALL, were very frequent and nearly exclusively found in ETP-ALL characterized by a specific immunophenotype. These molecular characteristics provide biologic insights and implications with respect to innovative treatment strategies (for example, tyrosine kinase inhibitors) for this high-risk subgroup of adult ETP-ALL.
KW  - acute lymphoblastic leukemia
KW  - ETP-ALL
KW  - T-ALL
KW  - molecular characteristics
KW  - FLT3
KW  - NOTCH1
Y1  - 2012
UR  - http://publikationen.ub.uni-frankfurt.de/frontdoor/index/index/docId/24462
UR  - https://nbn-resolving.org/urn:nbn:de:hebis:30:3-244627
SN  - 2044-5385
VL  - 2
IS  - e55
PB  - Nature Publishing Group
CY  - London [u.a.]
ER  -