PMID- 9389711
OWN - NLM
STAT- MEDLINE
DCOM- 19971229
LR  - 20231213
IS  - 0006-4971 (Print)
IS  - 0006-4971 (Linking)
VI  - 90
IP  - 12
DP  - 1997 Dec 15
TI  - Incidence of TEL/AML1 fusion gene analyzed consecutively in children with acute 
      lymphoblastic leukemia in relapse.
PG  - 4933-7
AB  - The translocation t(12; 21)(p13; q22) is difficult to detect by classic 
      cytogenetics. However, using fluorescence in situ hybridization (FISH) and by 
      screening for the TEL/AML1 rearrangement by the polymerase chain reaction (PCR), 
      it has been demonstrated to be the most frequent known structural chromosomal 
      abnormality in childhood acute lymphoblastic leukemia (ALL). It is closely 
      correlated with a B-cell precursor (BCP) phenotype and is considered a favorable 
      prognostic factor. However, little is known about the incidence of the 
      translocation in relapsed patients and the duration of complete remission (CR) in 
      children expressing the TEL/AML1 fusion gene. We therefore examined 49 bone 
      marrow samples from children with ALL at first or second relapse that were 
      consecutively mailed to our laboratory to test for the presence of t(12; 21) 
      using reverse transcriptase (RT)-PCR. The TEL/AML1 rearrangement could be 
      identified in nine of 44 (20%) of the patients, a result similar to the reported 
      incidence at diagnosis. Most of the TEL/AML1-positive children showed no adverse 
      clinical features at diagnosis (eg, white blood cell [WBC] count <100 x 10(9)/L 
      or age <10 years), and regarding these data, there were no differences versus 
      children who were negative for the fusion gene. However, the period of remission 
      was about 1 year longer in children expressing TEL/AML1 (P = .046), and the 
      majority of relapses in this group appeared late (<2 years after diagnosis). Our 
      findings therefore reinforce the urgent need for further prospective studies with 
      a long follow-up period to determine the true prognostic significance of t(12; 
      21) and to avoid premature changes of treatment strategies.
FAU - Harbott, J
AU  - Harbott J
AD  - Department of General Pediatrics, Hematology and Oncology, Children's University 
      Hospital, Giessen, Germany.
FAU - Viehmann, S
AU  - Viehmann S
FAU - Borkhardt, A
AU  - Borkhardt A
FAU - Henze, G
AU  - Henze G
FAU - Lampert, F
AU  - Lampert F
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Blood
JT  - Blood
JID - 7603509
RN  - 0 (Core Binding Factor Alpha 2 Subunit)
RN  - 0 (DNA-Binding Proteins)
RN  - 0 (Proto-Oncogene Proteins)
RN  - 0 (Proto-Oncogene Proteins c-ets)
RN  - 0 (RUNX1 protein, human)
RN  - 0 (Repressor Proteins)
RN  - 0 (Transcription Factors)
SB  - IM
MH  - Adolescent
MH  - Child
MH  - Child, Preschool
MH  - *Chromosomes, Human, Pair 12
MH  - *Chromosomes, Human, Pair 21
MH  - Core Binding Factor Alpha 2 Subunit
MH  - DNA-Binding Proteins/*genetics
MH  - Female
MH  - *Gene Rearrangement
MH  - Humans
MH  - Male
MH  - Precursor Cell Lymphoblastic Leukemia-Lymphoma/*genetics
MH  - Prognosis
MH  - *Proto-Oncogene Proteins
MH  - Proto-Oncogene Proteins c-ets
MH  - Recurrence
MH  - *Repressor Proteins
MH  - Transcription Factors/*genetics
MH  - *Translocation, Genetic
MH  - ETS Translocation Variant 6 Protein
EDAT- 1998/01/07 00:00
MHDA- 1998/01/07 00:01
CRDT- 1998/01/07 00:00
PHST- 1998/01/07 00:00 [pubmed]
PHST- 1998/01/07 00:01 [medline]
PHST- 1998/01/07 00:00 [entrez]
AID - S0006-4971(20)54958-6 [pii]
PST - ppublish
SO  - Blood. 1997 Dec 15;90(12):4933-7.