PMID- 29902576
OWN - NLM
STAT- MEDLINE
DCOM- 20190516
LR  - 20190516
IS  - 1532-8392 (Electronic)
IS  - 0046-8177 (Linking)
VI  - 80
DP  - 2018 Oct
TI  - Lymphomas with pseudo-double-hit BCL6-MYC translocations due to t(3;8)(q27;q24) 
      are associated with a germinal center immunophenotype, extranodal involvement, 
      and frequent BCL2 translocations.
PG  - 192-200
LID - S0046-8177(18)30212-0 [pii]
LID - 10.1016/j.humpath.2018.06.006 [doi]
AB  - High-grade B-cell lymphomas with MYC, BCL2, and/or BCL6 rearrangements, 
      "double-hit" or "triple-hit" lymphomas (DTHL), are aggressive neoplasms 
      associated with a poor prognosis. A t(3;8)(q27;q24) rarely occurs in B-cell 
      lymphomas that results in a unique "pseudo-double-hit" BCL6-MYC fusion, 
      indistinguishable by interphase fluorescence in situ hybridization (FISH) from 
      more conventional DTHL with independent MYC and BCL6 translocations. Reports of 
      t(3;8)(q27;q24) lymphomas are sparse, and to better characterize their 
      pathologic, cytogenetic, and clinical features, 6 new cases from 2 institutions 
      and 19 previously published cases were reviewed. All new cases displayed 
      aggressive morphologic features, and most previously published cases were 
      classified as aggressive lymphomas. Collectively, all t(3;8)(q27;q24) cases had a 
      germinal center (GC) phenotype, and most had complex karyotypes (22/24, 92%), 
      including frequent concomitant BCL2 rearrangements (17/24, 71%). When compared to 
      two large published DTHL cohorts, t(3;8)(q27;q24) lymphomas less often expressed 
      BCL2 (P < .01), had a greater likelihood of extranodal involvement (P < .01), and 
      more frequently appeared triple-hit by FISH analysis (P < .01). Despite 
      presenting with aggressive clinicopathologic features, 100% (6/6) of 
      t(3;8;)(q27;q24) patients achieved complete remission after intensive induction 
      regimens, and 2- and 3-year overall survival rates were 63% (10/16) and 57% 
      (8/14), respectively. These findings suggest that lymphomas with t(3;8)(q27;q24) 
      may represent a subset of GC B-cell lymphomas distinct from conventional DTHL. 
      Our results further highlight the value of routine karyotype assessment in 
      aggressive B-cell lymphomas, and the importance of recognizing the 
      t(3;8)(q27;q24) so that its clinical significance can be more fully explored.
CI  - Copyright (c) 2018 Elsevier Inc. All rights reserved.
FAU - Johnson, Steven M
AU  - Johnson SM
AD  - Department of Pathology and Laboratory Medicine, The University of North Carolina 
      School of Medicine, Chapel Hill, NC, 27599. Electronic address: 
      Steven.Johnson@unchealth.unc.edu.
FAU - Umakanthan, Jayadev Manikkam
AU  - Umakanthan JM
AD  - Department of Internal Medicine, The University of Nebraska Medical Center, 
      Omaha, NE 68198.
FAU - Yuan, Ji
AU  - Yuan J
AD  - Department of Pathology, The University of Nebraska Medical Center, Omaha, NE 
      68198.
FAU - Fedoriw, Yuri
AU  - Fedoriw Y
AD  - Department of Pathology and Laboratory Medicine, The University of North Carolina 
      School of Medicine, Chapel Hill, NC, 27599; Lineberger Comprehensive Cancer 
      Center, The University of North Carolina School of Medicine, Chapel Hill, NC 
      27599.
FAU - Bociek, R Gregory
AU  - Bociek RG
AD  - Department of Internal Medicine, The University of Nebraska Medical Center, 
      Omaha, NE 68198.
FAU - Kaiser-Rogers, Kathleen
AU  - Kaiser-Rogers K
AD  - Department of Pathology and Laboratory Medicine, The University of North Carolina 
      School of Medicine, Chapel Hill, NC, 27599; Departments of Pediatrics and 
      Genetics, The University of North Carolina School of Medicine, Chapel Hill, NC, 
      27514.
FAU - Sanmann, Jennifer N
AU  - Sanmann JN
AD  - Human Genetics Laboratory, Munroe-Meyer Institute, The University of Nebraska 
      Medical Center, Omaha, NE 68131.
FAU - Montgomery, Nathan D
AU  - Montgomery ND
AD  - Department of Pathology and Laboratory Medicine, The University of North Carolina 
      School of Medicine, Chapel Hill, NC, 27599; Lineberger Comprehensive Cancer 
      Center, The University of North Carolina School of Medicine, Chapel Hill, NC 
      27599.
LA  - eng
PT  - Journal Article
DEP - 20180611
PL  - United States
TA  - Hum Pathol
JT  - Human pathology
JID - 9421547
RN  - 0 (Proto-Oncogene Proteins c-bcl-2)
RN  - 0 (Proto-Oncogene Proteins c-bcl-6)
RN  - 0 (Proto-Oncogene Proteins c-myc)
SB  - IM
MH  - Adult
MH  - Aged
MH  - Female
MH  - Gene Rearrangement/*genetics
MH  - Germinal Center/*pathology
MH  - Humans
MH  - *Immunophenotyping
MH  - In Situ Hybridization, Fluorescence/methods
MH  - Lymphoma, B-Cell/pathology
MH  - Male
MH  - Middle Aged
MH  - Phenotype
MH  - Proto-Oncogene Proteins c-bcl-2/*genetics
MH  - Proto-Oncogene Proteins c-bcl-6/*genetics
MH  - Proto-Oncogene Proteins c-myc/genetics
MH  - Translocation, Genetic/genetics
OTO - NOTNLM
OT  - Double-hit lymphoma
OT  - High-grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements
OT  - Lymphoma cytogenetics
OT  - Triple-hit lymphoma
OT  - t(3;8)(q27;q24)
EDAT- 2018/06/15 06:00
MHDA- 2019/05/17 06:00
CRDT- 2018/06/15 06:00
PHST- 2018/04/11 00:00 [received]
PHST- 2018/05/23 00:00 [revised]
PHST- 2018/06/01 00:00 [accepted]
PHST- 2018/06/15 06:00 [pubmed]
PHST- 2019/05/17 06:00 [medline]
PHST- 2018/06/15 06:00 [entrez]
AID - S0046-8177(18)30212-0 [pii]
AID - 10.1016/j.humpath.2018.06.006 [doi]
PST - ppublish
SO  - Hum Pathol. 2018 Oct;80:192-200. doi: 10.1016/j.humpath.2018.06.006. Epub 2018 
      Jun 11.