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

Hematopathology

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

80 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 (US)
Pages (from-to)	1090-1098
Number of pages	9
Journal	Histopathology
Volume	68
Issue number	7
DOIs	https://doi.org/10.1111/his.12884
State	Published - Jun 1 2016

Keywords

BCL6/3q27
Double-hit lymphoma
MYC/8q24

ASJC Scopus subject areas

Pathology and Forensic Medicine
Histology

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10.1111/his.12884

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@article{5ed402101c1541cba53927a27d1c4ead,

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 = "BCL6/3q27, Double-hit lymphoma, MYC/8q24",

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 = "Publisher Copyright: {\textcopyright} 2016 John Wiley & Sons Ltd.",

year = "2016",

month = jun,

day = "1",

doi = "10.1111/his.12884",

language = "English (US)",

volume = "68",

pages = "1090--1098",

journal = "Histopathology",

issn = "0309-0167",

publisher = "Wiley-Blackwell",

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 - 2016/6/1

Y1 - 2016/6/1

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 - BCL6/3q27

KW - Double-hit lymphoma

KW - MYC/8q24

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U2 - 10.1111/his.12884

DO - 10.1111/his.12884

M3 - Article

C2 - 26426741

AN - SCOPUS:84949651479

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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