PMID- 29666005
OWN - NLM
STAT- MEDLINE
DCOM- 20190108
LR  - 20190108
IS  - 2210-7762 (Print)
VI  - 222-223
DP  - 2018 Apr
TI  - Unexpected favorable outcome in a patient with high grade B-cell lymphoma with 
      abnormalities of MYC, BCL6 and BCL2 loci.
PG  - 25-31
LID - S2210-7762(17)30299-5 [pii]
LID - 10.1016/j.cancergen.2018.01.003 [doi]
AB  - High grade B-cell lymphoma (HGBCL) by WHO 2016 classification requires 
      rearrangements of MYC and BCL2 and/or BCL6, practically covering the so called 
      "double-hit" or "triple hit" lymphomas. We report a case of HGBCL "triple-hit" 
      lymphoma in a 64-year old female. Cytogenetic and fluorescence in situ 
      hybridization (FISH) studies revealed complex karyotype including rearrangement 
      of MYC to a novel, non-IG partner on chromosome 18, and rearrangement of BCL2, 
      BCL6 and IGH as well as ins(3)(q21q27.3q25.1) among other abnormalities. FISH 
      studies showed five copies of MYC and 3-8 copies of BCL2. Gene expression 
      analysis by RNA sequencing showed that MYC, BCL2 and MECOM genes were 
      overexpressed whereas BCL6 was under-expressed. BCL6 was fused to MBNL1 gene due 
      to complex structural rearrangement. MYC was expressed in >70% of cells and BCL2 
      was diffusely but highly expressed by immunohistochemistry. No pathogenic 
      mutations were identified by sequencing a 26-gene panel including TP53. The 
      patient has unexpectedly been in complete remission for 12 months after diagnosis 
      after intensive chemotherapy including DA-EPOCH regimen despite having HGBCL. The 
      prognostication of HGBCL patients may further be improved by the 
      sub-categorization of these lymphomas on the basis of more detailed genomic 
      markers than merely the WHO 2016 classification.
CI  - Copyright (c) 2018 Elsevier Inc. All rights reserved.
FAU - Adams, Thomas
AU  - Adams T
AD  - University of Arizona, Banner University Medical Center, United States.
FAU - Fuchs, Deborah
AU  - Fuchs D
AD  - University of Arizona, Banner University Medical Center, United States.
FAU - Shadoan, Patricia K
AU  - Shadoan PK
AD  - Cytogenetic Laboratory at Banner University Medical Center, United States.
FAU - Johnstone, Laurel
AU  - Johnstone L
AD  - University of Arizona Genetic Core for Clinical Services Laboratory, Tucson, AZ, 
      United States.
FAU - Lau, Branden M
AU  - Lau BM
AD  - University of Arizona Genetic Core for Clinical Services Laboratory, Tucson, AZ, 
      United States.
FAU - McGhan, Lee
AU  - McGhan L
AD  - University of Arizona, Banner University Medical Center, United States.
FAU - Anwer, Faiz
AU  - Anwer F
AD  - University of Arizona, Banner University Medical Center, United States; 
      University of Arizona Cancer Center, United States.
FAU - Al-Kateb, Hussam
AU  - Al-Kateb H
AD  - University of Arizona, Banner University Medical Center, United States; 
      Cytogenetic Laboratory at Banner University Medical Center, United States; 
      University of Arizona Genetic Core for Clinical Services Laboratory, Tucson, AZ, 
      United States. Electronic address: halkateb@pathology.arizona.edu.
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20180203
PL  - United States
TA  - Cancer Genet
JT  - Cancer genetics
JID - 101539150
RN  - 0 (MDS1 and EVI1 Complex Locus Protein)
RN  - 0 (MECOM protein, human)
RN  - 0 (Proto-Oncogene Proteins c-bcl-2)
RN  - 0 (Proto-Oncogene Proteins c-bcl-6)
RN  - 0 (Proto-Oncogene Proteins c-myc)
RN  - 5J49Q6B70F (Vincristine)
RN  - 6PLQ3CP4P3 (Etoposide)
RN  - 80168379AG (Doxorubicin)
RN  - 8N3DW7272P (Cyclophosphamide)
RN  - VB0R961HZT (Prednisone)
RN  - EPOCH protocol
SB  - IM
MH  - Antineoplastic Combined Chemotherapy Protocols/therapeutic use
MH  - *Chromosome Inversion
MH  - Chromosomes, Human, Pair 18/genetics
MH  - Chromosomes, Human, Pair 3/genetics
MH  - Cyclophosphamide/therapeutic use
MH  - Doxorubicin/therapeutic use
MH  - Etoposide/therapeutic use
MH  - Female
MH  - Gene Expression Profiling
MH  - Gene Order
MH  - *Genes, myc
MH  - Humans
MH  - In Situ Hybridization, Fluorescence
MH  - Lymphoma, B-Cell/drug therapy/*genetics/*pathology
MH  - MDS1 and EVI1 Complex Locus Protein/genetics
MH  - Middle Aged
MH  - Neoplasm Grading
MH  - Prednisone/therapeutic use
MH  - Proto-Oncogene Proteins c-bcl-2/*genetics
MH  - Proto-Oncogene Proteins c-bcl-6/*genetics
MH  - Proto-Oncogene Proteins c-myc/*genetics
MH  - Remission Induction
MH  - Sequence Analysis, RNA
MH  - *Translocation, Genetic
MH  - Treatment Outcome
MH  - Vincristine/therapeutic use
OTO - NOTNLM
OT  - Double hit lymphoma
OT  - High grade B-cell lymphoma
OT  - MYC translocation
OT  - MYC translocation to non-IG
OT  - Prognostication of HGBCL
OT  - Triple hit lymphoma
EDAT- 2018/04/19 06:00
MHDA- 2019/01/09 06:00
CRDT- 2018/04/19 06:00
PHST- 2017/07/13 00:00 [received]
PHST- 2018/01/18 00:00 [revised]
PHST- 2018/01/24 00:00 [accepted]
PHST- 2018/04/19 06:00 [entrez]
PHST- 2018/04/19 06:00 [pubmed]
PHST- 2019/01/09 06:00 [medline]
AID - S2210-7762(17)30299-5 [pii]
AID - 10.1016/j.cancergen.2018.01.003 [doi]
PST - ppublish
SO  - Cancer Genet. 2018 Apr;222-223:25-31. doi: 10.1016/j.cancergen.2018.01.003. Epub 
      2018 Feb 3.