PMID- 11106814
OWN - NLM
STAT- MEDLINE
DCOM- 20001222
LR  - 20190816
IS  - 0165-4608 (Print)
IS  - 0165-4608 (Linking)
VI  - 122
IP  - 2
DP  - 2000 Oct 15
TI  - TEL/AML-1 fusion gene. its frequency and prognostic significance in childhood 
      acute lymphoblastic leukemia.
PG  - 73-8
AB  - TEL gene rearrangement due to the 12;21 chromosome translocation is believed to 
      be the most common molecular genetic abnormality in childhood acute lymphoblastic 
      leukemia (ALL). A study was conducted to investigate the frequency and prognostic 
      significance of TEL/AML-1 fusion gene resulting from a cryptic t(12;21)(p13;q22). 
      Bone marrow samples from 86 patients diagnosed over the past 5 years at Columbus 
      Children's Hospital were analyzed by fluorescence in situ hybridization (FISH) 
      technique for TEL/AML-1 fusion gene, using LSI((R)) DNA probes. The positive 
      cases were analyzed for clinical outcome. Patients in this study received 
      treatment according to Children's Cancer Group (CCG) protocols. Fifteen of the 86 
      cases (17%) were positive for the fusion gene. All were B-cell lineage and except 
      for one, all were CD10 positive. TEL/AML-1 was not found in any T-cell ALL. The 
      mean overall survival (OS) following diagnosis for the TEL/AML-1-positive group 
      was significantly longer than for the TEL/AML-1-negative group by log-rank = 
      7.84, P = 0.005. Similarly, the event-free survival (EFS) after remission for the 
      positive group (median 94.5 months) was longer than the negative group (median 57 
      months) by log-rank = 7.19, P = 0.007. This study confirms that the TEL/AML-1 
      fusion gene may be the most common genetic event in childhood ALL, occurring in 
      17% of the patients. It appears restricted to the B-cell lineage. In this study, 
      the presence of a TEL/AML-1 fusion gene was statistically significant in 
      predicting both OS and EFS, indicating a favorable clinical outcome for these 
      patients. Screening for TEL/AML-1 should become routine at diagnosis and a useful 
      biological variable for risk stratification in future clinical trials.
FAU - Jamil, A
AU  - Jamil A
AD  - Department of Pediatrics, Division of Hematology/Oncology, Children's Hospital, 
      700 Children's Drive, Columbus, OH 43205, USA. jamila@pediatrics.ohio-state.edu
FAU - Theil, K S
AU  - Theil KS
FAU - Kahwash, S
AU  - Kahwash S
FAU - Ruymann, F B
AU  - Ruymann FB
FAU - Klopfenstein, K J
AU  - Klopfenstein KJ
LA  - eng
GR  - P30CA16058/CA/NCI NIH HHS/United States
GR  - U10CA03750-39/CA/NCI NIH HHS/United States
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - United States
TA  - Cancer Genet Cytogenet
JT  - Cancer genetics and cytogenetics
JID - 7909240
RN  - 0 (Core Binding Factor Alpha 2 Subunit)
RN  - 0 (Oncogene Proteins, Fusion)
RN  - 0 (TEL-AML1 fusion protein)
SB  - IM
MH  - Antineoplastic Combined Chemotherapy Protocols/therapeutic use
MH  - Bone Marrow Cells/cytology/metabolism
MH  - Child
MH  - Child, Preschool
MH  - Core Binding Factor Alpha 2 Subunit
MH  - Female
MH  - Gene Frequency
MH  - Humans
MH  - In Situ Hybridization
MH  - In Situ Hybridization, Fluorescence
MH  - Infant
MH  - Infant, Newborn
MH  - Male
MH  - Oncogene Proteins, Fusion/*genetics
MH  - Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug therapy/*genetics/pathology
MH  - Prognosis
MH  - Survival Analysis
EDAT- 2000/12/07 11:00
MHDA- 2001/02/28 10:01
CRDT- 2000/12/07 11:00
PHST- 2000/12/07 11:00 [pubmed]
PHST- 2001/02/28 10:01 [medline]
PHST- 2000/12/07 11:00 [entrez]
AID - S0165-4608(00)00272-7 [pii]
AID - 10.1016/s0165-4608(00)00272-7 [doi]
PST - ppublish
SO  - Cancer Genet Cytogenet. 2000 Oct 15;122(2):73-8. doi: 
      10.1016/s0165-4608(00)00272-7.