MYC/BCL6 double-hit lymphoma (DHL): a tumour associated with an aggressive clinical course and poor prognosis

Shaoying Li; Parth Desai; Pei Lin; C Cameron Yin; Guilin Tang; Xuan J Wang; Sergej N Konoplev; Joseph D Khoury; Carlos E Bueso-Ramos; L Jeffrey Medeiros

doi:10.1111/his.12884

MYC/BCL6 double-hit lymphoma (DHL): a tumour associated with an aggressive clinical course and poor prognosis

Shaoying Li, Parth Desai, Pei Lin, C Cameron Yin, Guilin Tang, Xuan J Wang, Sergej N Konoplev, Joseph D Khoury, Carlos E Bueso-Ramos, L Jeffrey Medeiros

Research output: Contribution to journal › Article › peer-review

95 Scopus citations

Abstract

AIMS: Large B cell lymphomas with MYC and BCL6/3q27 rearrangements, designated MYC/BCL6 DHL, are uncommon. Our aim was to better characterize this group of tumours.

METHODS AND RESULTS: We studied the clinicopathological features and outcome of 13 patients with MYC/BCL6 DHL and compared this group to a group of 83 MYC/BCL2 DHL patients. There were eight men and five women, with a median age of 63 years. Eleven tumours were classified as diffuse large B cell lymphomas (DLBCL) and two were B cell lymphoma, unclassifiable, with features intermediate between DLBCL and Burkitt lymphoma (BCLU). Immunohistochemical analysis showed that these tumours were positive for BCL6 (100%), BCL2 (eight of 10; 80%) and CD10 (eight of 10; 80%). Nine of 12 (75%) cases had a germinal centre B cell (GCB) immunophenotype; in one case data were incomplete. All patients were treated with chemotherapy. The clinicopathological features of MYC/BCL6 DHL were similar to MYC/BCL2 DHL, except that MYC/BCL6 DHL had a GCB immunophenotype less often. Patients with MYC/BCL6 DHL had a poor overall survival, similar to patients with MYC/BCL2 DHL (P = 0.32).

CONCLUSIONS: MYC/BCL6 DHL is an aggressive B cell lymphoma and patients often have an aggressive clinical course and poor prognosis, similar to patients with MYC/BCL2 DHL.

Original language	English
Pages (from-to)	1090-8
Number of pages	9
Journal	Histopathology
Volume	68
Issue number	7
DOIs	https://doi.org/10.1111/his.12884
State	Published - Jun 2016
Externally published	Yes

Keywords

Adult
Aged
Aged, 80 and over
B-Lymphocytes/metabolism
Biomarkers, Tumor/genetics
Burkitt Lymphoma/classification
Female
Gene Rearrangement
Germinal Center/metabolism
Humans
Immunophenotyping
In Situ Hybridization, Fluorescence
Lymphoma, B-Cell/classification
Lymphoma, Large B-Cell, Diffuse/classification
Male
Middle Aged
Prognosis
Proto-Oncogene Proteins c-bcl-6/genetics
Proto-Oncogene Proteins c-myc/genetics

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10.1111/his.12884

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@article{edf1808bb0f54c2e971d76af1c806b68,

title = "MYC/BCL6 double-hit lymphoma (DHL): a tumour associated with an aggressive clinical course and poor prognosis",

abstract = "AIMS: Large B cell lymphomas with MYC and BCL6/3q27 rearrangements, designated MYC/BCL6 DHL, are uncommon. Our aim was to better characterize this group of tumours.METHODS AND RESULTS: We studied the clinicopathological features and outcome of 13 patients with MYC/BCL6 DHL and compared this group to a group of 83 MYC/BCL2 DHL patients. There were eight men and five women, with a median age of 63 years. Eleven tumours were classified as diffuse large B cell lymphomas (DLBCL) and two were B cell lymphoma, unclassifiable, with features intermediate between DLBCL and Burkitt lymphoma (BCLU). Immunohistochemical analysis showed that these tumours were positive for BCL6 (100\%), BCL2 (eight of 10; 80\%) and CD10 (eight of 10; 80\%). Nine of 12 (75\%) cases had a germinal centre B cell (GCB) immunophenotype; in one case data were incomplete. All patients were treated with chemotherapy. The clinicopathological features of MYC/BCL6 DHL were similar to MYC/BCL2 DHL, except that MYC/BCL6 DHL had a GCB immunophenotype less often. Patients with MYC/BCL6 DHL had a poor overall survival, similar to patients with MYC/BCL2 DHL (P = 0.32).CONCLUSIONS: MYC/BCL6 DHL is an aggressive B cell lymphoma and patients often have an aggressive clinical course and poor prognosis, similar to patients with MYC/BCL2 DHL.",

keywords = "Adult, Aged, Aged, 80 and over, B-Lymphocytes/metabolism, Biomarkers, Tumor/genetics, Burkitt Lymphoma/classification, Female, Gene Rearrangement, Germinal Center/metabolism, Humans, Immunophenotyping, In Situ Hybridization, Fluorescence, Lymphoma, B-Cell/classification, Lymphoma, Large B-Cell, Diffuse/classification, Male, Middle Aged, Prognosis, Proto-Oncogene Proteins c-bcl-6/genetics, Proto-Oncogene Proteins c-myc/genetics",

author = "Shaoying Li and Parth Desai and Pei Lin and Yin, \{C Cameron\} and Guilin Tang and Wang, \{Xuan J\} and Konoplev, \{Sergej N\} and Khoury, \{Joseph D\} and Bueso-Ramos, \{Carlos E\} and Medeiros, \{L Jeffrey\}",

note = "{\textcopyright} 2015 John Wiley \& Sons Ltd.",

year = "2016",

month = jun,

doi = "10.1111/his.12884",

language = "English",

volume = "68",

pages = "1090--8",

journal = "Histopathology",

issn = "0309-0167",

publisher = "Wiley-Blackwell Publishing Ltd",

number = "7",

}

TY - JOUR

T1 - MYC/BCL6 double-hit lymphoma (DHL)

T2 - a tumour associated with an aggressive clinical course and poor prognosis

AU - Li, Shaoying

AU - Desai, Parth

AU - Lin, Pei

AU - Yin, C Cameron

AU - Tang, Guilin

AU - Wang, Xuan J

AU - Konoplev, Sergej N

AU - Khoury, Joseph D

AU - Bueso-Ramos, Carlos E

AU - Medeiros, L Jeffrey

PY - 2016/6

Y1 - 2016/6

N2 - AIMS: Large B cell lymphomas with MYC and BCL6/3q27 rearrangements, designated MYC/BCL6 DHL, are uncommon. Our aim was to better characterize this group of tumours.METHODS AND RESULTS: We studied the clinicopathological features and outcome of 13 patients with MYC/BCL6 DHL and compared this group to a group of 83 MYC/BCL2 DHL patients. There were eight men and five women, with a median age of 63 years. Eleven tumours were classified as diffuse large B cell lymphomas (DLBCL) and two were B cell lymphoma, unclassifiable, with features intermediate between DLBCL and Burkitt lymphoma (BCLU). Immunohistochemical analysis showed that these tumours were positive for BCL6 (100%), BCL2 (eight of 10; 80%) and CD10 (eight of 10; 80%). Nine of 12 (75%) cases had a germinal centre B cell (GCB) immunophenotype; in one case data were incomplete. All patients were treated with chemotherapy. The clinicopathological features of MYC/BCL6 DHL were similar to MYC/BCL2 DHL, except that MYC/BCL6 DHL had a GCB immunophenotype less often. Patients with MYC/BCL6 DHL had a poor overall survival, similar to patients with MYC/BCL2 DHL (P = 0.32).CONCLUSIONS: MYC/BCL6 DHL is an aggressive B cell lymphoma and patients often have an aggressive clinical course and poor prognosis, similar to patients with MYC/BCL2 DHL.

AB - AIMS: Large B cell lymphomas with MYC and BCL6/3q27 rearrangements, designated MYC/BCL6 DHL, are uncommon. Our aim was to better characterize this group of tumours.METHODS AND RESULTS: We studied the clinicopathological features and outcome of 13 patients with MYC/BCL6 DHL and compared this group to a group of 83 MYC/BCL2 DHL patients. There were eight men and five women, with a median age of 63 years. Eleven tumours were classified as diffuse large B cell lymphomas (DLBCL) and two were B cell lymphoma, unclassifiable, with features intermediate between DLBCL and Burkitt lymphoma (BCLU). Immunohistochemical analysis showed that these tumours were positive for BCL6 (100%), BCL2 (eight of 10; 80%) and CD10 (eight of 10; 80%). Nine of 12 (75%) cases had a germinal centre B cell (GCB) immunophenotype; in one case data were incomplete. All patients were treated with chemotherapy. The clinicopathological features of MYC/BCL6 DHL were similar to MYC/BCL2 DHL, except that MYC/BCL6 DHL had a GCB immunophenotype less often. Patients with MYC/BCL6 DHL had a poor overall survival, similar to patients with MYC/BCL2 DHL (P = 0.32).CONCLUSIONS: MYC/BCL6 DHL is an aggressive B cell lymphoma and patients often have an aggressive clinical course and poor prognosis, similar to patients with MYC/BCL2 DHL.

KW - Adult

KW - Aged

KW - Aged, 80 and over

KW - B-Lymphocytes/metabolism

KW - Biomarkers, Tumor/genetics

KW - Burkitt Lymphoma/classification

KW - Female

KW - Gene Rearrangement

KW - Germinal Center/metabolism

KW - Humans

KW - Immunophenotyping

KW - In Situ Hybridization, Fluorescence

KW - Lymphoma, B-Cell/classification

KW - Lymphoma, Large B-Cell, Diffuse/classification

KW - Male

KW - Middle Aged

KW - Prognosis

KW - Proto-Oncogene Proteins c-bcl-6/genetics

KW - Proto-Oncogene Proteins c-myc/genetics

U2 - 10.1111/his.12884

DO - 10.1111/his.12884

M3 - Article

C2 - 26426741

SN - 0309-0167

VL - 68

SP - 1090

EP - 1098

JO - Histopathology

JF - Histopathology

IS - 7

ER -

MYC/BCL6 double-hit lymphoma (DHL): a tumour associated with an aggressive clinical course and poor prognosis

Abstract

Keywords

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