PMID- 19998443
OWN - NLM
STAT- MEDLINE
DCOM- 20100224
LR  - 20231213
IS  - 1098-2264 (Electronic)
IS  - 1045-2257 (Linking)
VI  - 49
IP  - 3
DP  - 2010 Mar
TI  - Cytogenetics of long-term survivors of ETV6-RUNX1 fusion positive acute 
      lymphoblastic leukemia.
PG  - 253-9
LID - 10.1002/gcc.20736 [doi]
AB  - This study describes the cytogenetics of 33 children with ETV6-RUNX1 positive 
      acute lymphoblastic leukemia (ALL) who had been in continuous complete remission 
      for a minimum of 8.8 years [median event-free survival (EFS) 10.9 years]. The 
      results were compared with a published series of 16 fusion positive patients 
      treated on the same childhood ALL trial, who had relapsed (median EFS, 2.3 
      years). Interphase fluorescence in situ hybridization (FISH) at diagnosis showed 
      deletion of the second ETV6 signal from all fusion positive cells in 45% of the 
      long-term survivors but in none of the relapsed patients, whereas patients with 
      mixed populations with retained or lost second signals were more frequent among 
      those who had relapsed (69%) than the long-term survivors (21%). Interphase 
      populations with two fusion signals in 18% of the long-term survivors and 31% of 
      relapsed patients were smaller in the long-term survivors (median, 4% of total 
      cells) than in the relapsed patients (median, 84%). The additional copy of 
      chromosome 21 in 30% of long-term survivors and in 69% of relapsed patients was a 
      derived chromosome 21 in 20% and 55% of patients, respectively. Metaphase FISH 
      for 26 long-term survivors and 15 relapsed patients revealed complex karyotypes 
      in both groups. Variant translocations involved different chromosome arms between 
      the long-term survivors and relapsed patients. It appears that the two groups 
      have some distinguishing cytogenetic features at the time of diagnosis, which may 
      provide pointers to relapse that are worthy of more detailed study.
FAU - Konn, Zoe J
AU  - Konn ZJ
AD  - Leukaemia Research Cytogenetics Group, Cancer Sciences Division, University of 
      Southampton, Southampton, UK.
FAU - Martineau, Mary
AU  - Martineau M
FAU - Bown, Nicholas
AU  - Bown N
FAU - Richards, Sue
AU  - Richards S
FAU - Swansbury, John
AU  - Swansbury J
FAU - Talley, Polly
AU  - Talley P
FAU - Wright, Sarah L
AU  - Wright SL
FAU - Harrison, Christine J
AU  - Harrison CJ
LA  - eng
GR  - MC_U137686856/MRC_/Medical Research Council/United Kingdom
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Genes Chromosomes Cancer
JT  - Genes, chromosomes & cancer
JID - 9007329
RN  - 0 (Core Binding Factor Alpha 2 Subunit)
RN  - 0 (Proto-Oncogene Proteins c-ets)
RN  - 0 (RUNX1 protein, human)
RN  - 0 (Repressor Proteins)
SB  - IM
MH  - Adolescent
MH  - Child
MH  - Child, Preschool
MH  - Core Binding Factor Alpha 2 Subunit/*genetics
MH  - Follow-Up Studies
MH  - *Gene Fusion
MH  - Humans
MH  - In Situ Hybridization, Fluorescence
MH  - Precursor B-Cell Lymphoblastic Leukemia-Lymphoma/*genetics/pathology
MH  - Proto-Oncogene Proteins c-ets/*genetics
MH  - Recurrence
MH  - Repressor Proteins/*genetics
MH  - *Survivors
MH  - Time Factors
MH  - ETS Translocation Variant 6 Protein
EDAT- 2009/12/10 06:00
MHDA- 2010/02/25 06:00
CRDT- 2009/12/10 06:00
PHST- 2009/12/10 06:00 [entrez]
PHST- 2009/12/10 06:00 [pubmed]
PHST- 2010/02/25 06:00 [medline]
AID - 10.1002/gcc.20736 [doi]
PST - ppublish
SO  - Genes Chromosomes Cancer. 2010 Mar;49(3):253-9. doi: 10.1002/gcc.20736.