PMID- 16763978
OWN - NLM
STAT- MEDLINE
DCOM- 20070511
LR  - 20231213
IS  - 1545-5009 (Print)
IS  - 1545-5009 (Linking)
VI  - 48
IP  - 5
DP  - 2007 May
TI  - Characteristics of patients with TEL-AML1-positive acute lymphoblastic leukemia 
      with single or multiple fusions.
PG  - 510-4
AB  - BACKGROUND: The TEL-AML1 fusion in precursor-B ALL is generated by a cryptic 
      12;21 translocation that is detectable by fluorescence in situ hybridization 
      (FISH). It is generally considered a favorable prognostic indicator. Some 
      TEL-AML1+ ALL patients present at diagnosis with extra copies of the fusion, 
      enumerated by FISH. The aim of the study was to determine whether additional 
      copies of TEL-AML1 have clinical significance. PROCEDURE: Charts of all TEL-AML1+ 
      ALL patients at the UM and Children's Hospitals and Clinics of Minnesota between 
      1996 and 2004 were reviewed. RESULTS: Eight patients (7 males/1 female, mean age 
      46 months) with two or more TEL-AML1 fusion signals and 24 with single TEL-AML1 
      fusion signals (18 males/6 females, mean age 52 months) were identified. There 
      was no statistically significant difference in age or gender between the two 
      groups. Patients with double TEL-AML1+ had a higher frequency of myeloid markers 
      CD13 (P = 0.04) or CD33 (P = 0.003) than single TEL-AML1+ patients. Single 
      TEL-AML1+ patients had higher WBC (P = 0.04) than double TEL-AML1+ patients. A 
      trend toward slower therapy response was seen in double TEL-AML1+ patients versus 
      single, (1 of 7 [14%] <5% marrow blasts on Day 7 vs. 13 of 23 [56%], P = 0.09). 
      Double TEL-AML1+ patients had a higher relapse rate (P = 0.09) than single 
      TEL-AML1+ patients. CONCLUSIONS: Utilizing FISH to distinguish subgroups of 
      TEL-AML1 fusion patients may have important prognostic implications. The presence 
      of an extra fusion may portend poorer prognosis. A larger and longer-term 
      follow-up study will be required to verify the possible clinical significance of 
      the presence of multiple TEL-AML1 fusions.
FAU - Al-Sweedan, Suleimman A
AU  - Al-Sweedan SA
AD  - Department of Pediatrics, University of Minnesota, Minneapolis, Minnesota 55455, 
      USA.
FAU - Neglia, Joseph P
AU  - Neglia JP
FAU - Steiner, Marie E
AU  - Steiner ME
FAU - Bostrom, Bruce C
AU  - Bostrom BC
FAU - Casey, Timothy
AU  - Casey T
FAU - Hirsch, Betsy A
AU  - Hirsch BA
LA  - eng
PT  - Comparative Study
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Pediatr Blood Cancer
JT  - Pediatric blood & cancer
JID - 101186624
RN  - 0 (Antigens, CD)
RN  - 0 (Antigens, Differentiation, Myelomonocytic)
RN  - 0 (CD33 protein, human)
RN  - 0 (Core Binding Factor Alpha 2 Subunit)
RN  - 0 (Proto-Oncogene Proteins c-ets)
RN  - 0 (RUNX1 protein, human)
RN  - 0 (Repressor Proteins)
RN  - 0 (Sialic Acid Binding Ig-like Lectin 3)
RN  - EC 3.4.11.2 (CD13 Antigens)
SB  - IM
MH  - Antigens, CD/analysis
MH  - Antigens, Differentiation, Myelomonocytic/analysis
MH  - CD13 Antigens/analysis
MH  - Child
MH  - Child, Preschool
MH  - Chromosomes, Human, 21-22 and Y
MH  - Chromosomes, Human, 6-12 and X
MH  - Core Binding Factor Alpha 2 Subunit/*genetics
MH  - Female
MH  - Humans
MH  - In Situ Hybridization, Fluorescence
MH  - Infant
MH  - Leukocyte Count
MH  - Male
MH  - Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug therapy/*genetics
MH  - Prognosis
MH  - Proto-Oncogene Proteins c-ets/*genetics
MH  - Repressor Proteins/*genetics
MH  - Retrospective Studies
MH  - Sialic Acid Binding Ig-like Lectin 3
MH  - Translocation, Genetic
MH  - ETS Translocation Variant 6 Protein
EDAT- 2006/06/10 09:00
MHDA- 2007/05/12 09:00
CRDT- 2006/06/10 09:00
PHST- 2006/06/10 09:00 [pubmed]
PHST- 2007/05/12 09:00 [medline]
PHST- 2006/06/10 09:00 [entrez]
AID - 10.1002/pbc.20911 [doi]
PST - ppublish
SO  - Pediatr Blood Cancer. 2007 May;48(5):510-4. doi: 10.1002/pbc.20911.