PMID- 18024371
OWN - NLM
STAT- MEDLINE
DCOM- 20071213
LR  - 20220408
IS  - 1592-8721 (Electronic)
IS  - 0390-6078 (Linking)
VI  - 92
IP  - 10
DP  - 2007 Oct
TI  - The clinical presentation and prognosis of diffuse large B-cell lymphoma with 
      t(14;18) and 8q24/c-MYC rearrangement.
PG  - 1335-42
AB  - BACKGROUND AND OBJECTIVES: Diffuse large B-cell lymphomas (DLBCL) are common 
      lymphomas that have been classified into three subgroups on the basis of their 
      patterns of gene expression. The aim of this study was to characterize the 
      clinical, biological, immunophenotypic and cytogenetic features of DLBCL with 
      concurrent t(14;18) and 8q24/c-MYC rearrangement. DESIGN AND METHODS: Sixteen 
      cases of DLBCL with the dual translocation were identified between 1998 and 
      January 2006. The clinical features of these cases were examined and 
      morphological, immunohistochemical, flow cytometric and cytogenetic analyses were 
      performed. RESULTS: All patients had aggressive features: B symptoms (81%), ECOG 
      performance status >2 (81%), elevated lactate dehydrogenase (100%), stage IV 
      disease (100%) with at least one extra-nodal localization (bone marrow, blood and 
      central nervous system involvement in 93%, 50% and 50%, respectively) and 
      age-adjusted IPI score of 3 in 81%. Despite intensive chemotherapy regimens 
      (including allogeneic transplants), all patients died of disease progression. 
      Progression-free and overall survival was 4 and 5 months, respectively. 
      Immunophenotyping analysis (CD20, CD10, Bcl-6, Mum-1, Bcl-2 CD138, MIB1, CD19, 
      CD5, CD38 and sIg) was performed and showed DLBCL with a germinal center (GC) 
      profile. Ki-67 staining ranged from 70 to 90%. All cases assessed by cytogenetics 
      analysis [conventional cytogenetic and/or fluorescence in situ hybridization 
      (FISH)] had a complex karyotype. In one case, we identified a 8q24/c-MYC 
      translocation variant never reported in DLBCL before: t(8;9)(q24;p13) and 
      t(14;18)(q32;q21). The BCL-6 rearrangement was investigated by FISH and found to 
      rearranged in four cases. INTERPRETATION AND CONCLUSIONS: In conclusion, DLBCL 
      with concurrent t(14;18) and 8q24/c-MYC rearrangement is a subgroup of 
      GC-DLBCLwith poor outcome. It is worth searching for the coexistence of dual 
      translocations in Bcl-2-positive DLBCL with unusual aggressive presentation.
FAU - Le Gouill, Steven
AU  - Le Gouill S
AD  - Department of Hematology, Hotel Dieu, University Hospital of Nantes, Place Alexis 
      Ricordeau, 4093 Nantes cedex 01, France.
FAU - Talmant, Pascaline
AU  - Talmant P
FAU - Touzeau, Cyrille
AU  - Touzeau C
FAU - Moreau, Anne
AU  - Moreau A
FAU - Garand, Richard
AU  - Garand R
FAU - Juge-Morineau, Nadine
AU  - Juge-Morineau N
FAU - Gaillard, Fanny
AU  - Gaillard F
FAU - Gastinne, Thomas
AU  - Gastinne T
FAU - Milpied, Noel
AU  - Milpied N
FAU - Moreau, Philippe
AU  - Moreau P
FAU - Harousseau, Jean Luc
AU  - Harousseau JL
FAU - Avet-Loiseau, Herve
AU  - Avet-Loiseau H
LA  - eng
PT  - Journal Article
PL  - Italy
TA  - Haematologica
JT  - Haematologica
JID - 0417435
RN  - 0 (MYC protein, human)
RN  - 0 (Proto-Oncogene Proteins c-myc)
SB  - IM
CIN - Haematologica. 2007 Oct;92(10):1297-301. PMID: 18024366
CIN - Haematologica. 2008 Jul;93(7):e53; author reply e54. PMID: 18591613
MH  - Adult
MH  - Aged
MH  - Chromosomes, Human, Pair 8/*genetics
MH  - Female
MH  - Humans
MH  - Immunophenotyping
MH  - Lymphoma, Large B-Cell, Diffuse/*genetics/*pathology
MH  - Male
MH  - Middle Aged
MH  - Prognosis
MH  - Proto-Oncogene Proteins c-myc/*genetics
EDAT- 2007/11/21 09:00
MHDA- 2007/12/14 09:00
CRDT- 2007/11/21 09:00
PHST- 2007/11/21 09:00 [pubmed]
PHST- 2007/12/14 09:00 [medline]
PHST- 2007/11/21 09:00 [entrez]
AID - 92/10/1335 [pii]
AID - 10.3324/haematol.11305 [doi]
PST - ppublish
SO  - Haematologica. 2007 Oct;92(10):1335-42. doi: 10.3324/haematol.11305.