PMID- 9473238
OWN - NLM
STAT- MEDLINE
DCOM- 19980316
LR  - 20210216
IS  - 0006-4971 (Print)
IS  - 0006-4971 (Linking)
VI  - 91
IP  - 5
DP  - 1998 Mar 1
TI  - TEL-AML1 fusion transcript in relapsed childhood acute lymphoblastic leukemia. 
      The Berlin-Frankfurt-Munster Study Group.
PG  - 1716-22
AB  - The cryptic translocation t(12;21)(p13;q22) has been recently recognized as the 
      most common genetic rearrangement in B-lineage childhood acute lymphoblastic 
      leukemia (ALL). The resulting fusion transcript, termed TEL-AML1, has been 
      associated with an excellent prognosis at initial ALL diagnosis. Hence, we 
      postulated that the incidence of TEL-AML1 fusion should be lower in patients with 
      ALL relapse. To address this assumption and to investigate the prognostic 
      significance of TEL-AML1 expression in relapsed childhood ALL, bone marrow 
      samples of 146 children were analyzed by reverse-transcriptase (RT)-polymerase 
      chain reaction (PCR). All children were treated according to 
      Berlin-Frankfurt-Munster (BFM) ALL relapse trial protocols (ALL-REZ BFM 90-96). 
      Their clinical features and outcome were compared with those of 262 patients who 
      could not be tested due to lack of bone marrow samples. Thirty-two of 146 
      children with relapsed ALL were TEL-AML1-positive. Four of the negative patients 
      had T-lineage and nine Philadelphia chromosome (Ph)-positive leukemia. Thus, the 
      incidence of TEL-AML1 in relapsed Ph1-negative, B-cell precursor ALL is 32 of 133 
      (24%). The 32 TEL-AML1-positive and 101 negative patients differed significantly 
      with respect to duration of last remission (42.5 v 27 months; P = . 0001) and age 
      at initial diagnosis (53.5 v 74 months; P = .0269). At a median follow-up time of 
      21.5 months, children positive for TEL-AML1 had a significantly (P = .0011) 
      higher probability of event-free survival (EFS; 0.79 v 0.33). The predominant 
      majority of patients had been treated for initial ALL according to German 
      multicenter BFM (108 of 133) or Cooperative ALL study group (CoALL) (19 of 133) 
      frontline protocols. The comparison of tested and not-tested (N = 262) patients 
      showed no significant difference. TEL-AML1 positivity predicted a favorable 
      short-term outcome; long-term results are unknown. Screening for TEL-AML1 should 
      become routine at relapse diagnosis and might be used for therapy stratification 
      in future trials.
FAU - Seeger, K
AU  - Seeger K
AD  - Department of Pediatric Oncology/Hematology, Charit-e-Virchow-Klinikum, 
      Humboldt-University at Berlin, Berlin, Germany.
FAU - Adams, H P
AU  - Adams HP
FAU - Buchwald, D
AU  - Buchwald D
FAU - Beyermann, B
AU  - Beyermann B
FAU - Kremens, B
AU  - Kremens B
FAU - Niemeyer, C
AU  - Niemeyer C
FAU - Ritter, J
AU  - Ritter J
FAU - Schwabe, D
AU  - Schwabe D
FAU - Harms, D
AU  - Harms D
FAU - Schrappe, M
AU  - Schrappe M
FAU - Henze, G
AU  - Henze G
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 (Neoplasm Proteins)
RN  - 0 (Oncogene Proteins, Fusion)
RN  - 0 (RNA, Messenger)
RN  - 0 (TEL-AML1 fusion protein)
RN  - EC 2.7.7.49 (RNA-Directed DNA Polymerase)
SB  - IM
MH  - Adolescent
MH  - Bone Marrow/chemistry
MH  - Child
MH  - Child, Preschool
MH  - *Chromosomes, Human, Pair 12
MH  - *Chromosomes, Human, Pair 21
MH  - Core Binding Factor Alpha 2 Subunit
MH  - Disease-Free Survival
MH  - Humans
MH  - Neoplasm Proteins/*genetics
MH  - *Oncogene Proteins, Fusion
MH  - Polymerase Chain Reaction
MH  - Precursor Cell Lymphoblastic Leukemia-Lymphoma/*genetics/mortality/therapy
MH  - Prognosis
MH  - RNA, Messenger/*analysis
MH  - RNA-Directed DNA Polymerase
MH  - Recurrence
MH  - Remission Induction
MH  - *Translocation, Genetic
EDAT- 1998/03/21 00:00
MHDA- 1998/03/21 00:01
CRDT- 1998/03/21 00:00
PHST- 1998/03/21 00:00 [pubmed]
PHST- 1998/03/21 00:01 [medline]
PHST- 1998/03/21 00:00 [entrez]
AID - S0006-4971(20)57149-8 [pii]
PST - ppublish
SO  - Blood. 1998 Mar 1;91(5):1716-22.