PMID- 7583381
OWN - NLM
STAT- MEDLINE
DCOM- 19951207
LR  - 20190913
IS  - 1077-4114 (Print)
IS  - 1077-4114 (Linking)
VI  - 17
IP  - 4
DP  - 1995 Nov
TI  - Abnormalities of chromosome band 11q23 and the MLL gene in pediatric 
      myelomonocytic and monoblastic leukemias. Identification of the t(9;11) as an 
      indicator of long survival.
PG  - 277-83
AB  - PURPOSE AND METHODS: We reviewed the cytogenetic pattern of the malignant cells 
      in 36 patients who were < 20 years of age and who had M4 and M5 leukemias, 
      excluding M4Eo cases with inv(16). We performed fluorescence in situ 
      hybridization (FISH) and molecular studies to determine the actual incidence of 
      11q23/MLL abnormalities in these patients. RESULTS: Eighteen patients had 11q23 
      translocations or insertions detected by cytogenetic analysis (15 cases) or by 
      FISH (3 cases); 10 patients had t(9;11), all of whom had M5a. Eight patients had 
      other 11q23 translocations or insertions not involving chromosome 9[t(11q23)] 
      (four each had M4 or M5 leukemias). Eighteen cases with M4/M5 did not have 11q23 
      abnormalities. MLL rearrangements were found in all patients with translocations 
      or insertions of 11q23 who were studied. Clinically, children with t(9;11) were 
      indistinguishable from other patients with M4-M5 leukemias. In contrast, the 
      t(11q23) group was characterized by extreme hyperleukocytosis, CNS disease, and 
      skin involvement. Patients with the t(9;11) had a better outcome when compared 
      with patients in the t(11q23) group (EFS +/- SE at 3 years, 56 +/- 17% versus 10 
      +/- 10%, p = 0.04), and to all the remaining children with M4-M5 leukemias (p = 
      0.04). CONCLUSIONS: The combination of cytogenetic, FISH, and molecular analysis 
      provides a highly sensitive strategy for detection of 11q23/MLL gene 
      rearrangements in childhood M4-M5 leukemias. Our more precise classification of 
      these patients allows a more accurate correlation with outcome. The favorable 
      prognostic significance of the t(9;11) should be confirmed in prospective studies 
      including a larger number of children as well as adults.
FAU - Martinez-Climent, J A
AU  - Martinez-Climent JA
AD  - Department of Medicine, University of Chicago Medical Center, IL 60637, USA.
FAU - Espinosa, R 3rd
AU  - Espinosa R 3rd
FAU - Thirman, M J
AU  - Thirman MJ
FAU - Le Beau, M M
AU  - Le Beau MM
FAU - Rowley, J D
AU  - Rowley JD
LA  - eng
GR  - CA-40046/CA/NCI NIH HHS/United States
GR  - CA-42577/CA/NCI NIH HHS/United States
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Research Support, U.S. Gov't, Non-P.H.S.
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - United States
TA  - J Pediatr Hematol Oncol
JT  - Journal of pediatric hematology/oncology
JID - 9505928
RN  - 0 (DNA, Neoplasm)
RN  - 0 (DNA-Binding Proteins)
RN  - 0 (KMT2A protein, human)
RN  - 0 (Transcription Factors)
RN  - 149025-06-9 (Myeloid-Lymphoid Leukemia Protein)
RN  - EC 2.1.1.43 (Histone-Lysine N-Methyltransferase)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Child
MH  - Child, Preschool
MH  - Chromosome Banding
MH  - *Chromosomes, Human, Pair 11
MH  - *Chromosomes, Human, Pair 9
MH  - DNA, Neoplasm/analysis
MH  - DNA-Binding Proteins/*genetics
MH  - Female
MH  - Gene Rearrangement/genetics
MH  - Histone-Lysine N-Methyltransferase
MH  - Humans
MH  - In Situ Hybridization, Fluorescence
MH  - Infant
MH  - Leukemia, Monocytic, Acute/*genetics/mortality/pathology
MH  - Leukemia, Myelomonocytic, Acute/*genetics/mortality/pathology
MH  - Male
MH  - Myeloid-Lymphoid Leukemia Protein
MH  - *Proto-Oncogenes
MH  - Survival Analysis
MH  - *Transcription Factors
MH  - *Translocation, Genetic
EDAT- 1995/11/01 00:00
MHDA- 2001/03/28 10:01
CRDT- 1995/11/01 00:00
PHST- 1995/11/01 00:00 [pubmed]
PHST- 2001/03/28 10:01 [medline]
PHST- 1995/11/01 00:00 [entrez]
AID - 10.1097/00043426-199511000-00001 [doi]
PST - ppublish
SO  - J Pediatr Hematol Oncol. 1995 Nov;17(4):277-83. doi: 
      10.1097/00043426-199511000-00001.