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Primary mediastinal B-cell lymphoma: high frequency of BCL-6 mutations and consistent expression of the transcription factors OCT-2, BOB.1, and PU.1 in the absence of immunoglobulins

Primary mediastinal B-cell lymphoma: high frequency of BCL-6 mutations and consistent expression of the transcription factors OCT-2, BOB.1, and PU.1 in the absence of immunoglobulins
Primary mediastinal B-cell lymphoma: high frequency of BCL-6 mutations and consistent expression of the transcription factors OCT-2, BOB.1, and PU.1 in the absence of immunoglobulins
Although primary mediastinal (thymic) large B-cell lymphoma has been primarily studied, its precise phenotype, molecular characteristics, and histogenesis are still a matter of debate. The International Extranodal Lymphoma Study Group collected 137 such cases for extensive pathological review. Histologically, the lymphomatous growth was predominantly diffuse with fibrosis that induced compartmentalized cell aggregation. It consisted of large cells with varying degrees of nuclear polymorphism and clear to basophilic cytoplasm. On immunohistochemistry, the following phenotype was observed: CD45+, CD20+, CD79a+, PAX5/BSAP+, BOB.1+, Oct-2+, PU.1+, Bcl-2+, CD30+, HLA-DR+, MAL protein+/-, Bcl-6+/-, MUM1/IRF4+/-, CD10-/+, CD21-, CD15-, CD138-, CD68-, and CD3-. Immunoglobulins were negative both at immunohistochemistry and in situ hybridization. Molecular analysis, performed in 45 cases, showed novel findings. More than half of the cases displayed BCL-6 gene mutations, which usually occurred along with functioning somatic IgVH gene mutations and Bcl-6 and/or MUM1/IRF4 expression. The present study supports the concept that a sizable fraction of cases of this lymphoma are from activated germinal center or postgerminal center cells. However, it differs from other aggressive B-cell lymphomas in that it shows defective immunoglobulin production despite the expression of OCT-2, BOB.1, and PU.1 transcription factors and the lack of IgVH gene crippling mutations.
0002-9440
243-253
Pileri, Stefano A.
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Gaidano, Gianluca
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Zinzani, Pier Luigi
c8a817bb-0f54-4bde-90b2-d3339ed8fff5
Falini, Brunangelo
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Gaulard, Philippe
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Zucca, Emanuele
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Pieri, Federica
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Berra, Eva
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Sabattini, Elena
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Ascani, Stefano
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Piccioli, Milena
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Johnson, Peter W.m.
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Giardini, Roberto
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Pescarmona, Edoardo
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Novero, Domenico
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Piccaluga, Pier Paolo
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Marafioti, Teresa
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Alonso, Miguel A.
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Cavalli, Franco
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Pileri, Stefano A.
975a0cc0-1b1c-4bdf-bc94-63cce6c6b13c
Gaidano, Gianluca
1a6a7107-107b-4891-82e0-5fedadd2f65a
Zinzani, Pier Luigi
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Falini, Brunangelo
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Gaulard, Philippe
31bac8f3-32b6-4720-b659-f769d6e25c3b
Zucca, Emanuele
40bd5950-d74e-4169-a2ea-0bfc1058058e
Pieri, Federica
523081b6-ee55-46eb-a185-40b56d79d3e4
Berra, Eva
717928c7-2ad6-427c-9322-947df427d479
Sabattini, Elena
549eaa74-b2d8-47e1-88d1-de144738d04f
Ascani, Stefano
7d9a9d22-8986-476b-94b5-33361624301b
Piccioli, Milena
46355259-1055-4bf3-8de3-3513e4b6b766
Johnson, Peter W.m.
1a86c9a9-25b4-4529-9353-d14fd062d63b
Giardini, Roberto
ba000d94-fe1f-403a-b7cd-30cd5d1eb5cf
Pescarmona, Edoardo
7d11d192-6886-4a19-a462-8631e6c68193
Novero, Domenico
cf870346-f6c5-4222-a3ec-8ff213a3a68d
Piccaluga, Pier Paolo
26e64e8b-fb36-4f2a-bb2c-dcd2983bc331
Marafioti, Teresa
2036b6e2-1157-4170-a533-a7c2307d619b
Alonso, Miguel A.
db4c63dd-7d98-40a3-88c4-66893ddaf223
Cavalli, Franco
ce699517-7deb-4bad-8b14-afe749884b9e

Pileri, Stefano A., Gaidano, Gianluca, Zinzani, Pier Luigi, Falini, Brunangelo, Gaulard, Philippe, Zucca, Emanuele, Pieri, Federica, Berra, Eva, Sabattini, Elena, Ascani, Stefano, Piccioli, Milena, Johnson, Peter W.m., Giardini, Roberto, Pescarmona, Edoardo, Novero, Domenico, Piccaluga, Pier Paolo, Marafioti, Teresa, Alonso, Miguel A. and Cavalli, Franco (2003) Primary mediastinal B-cell lymphoma: high frequency of BCL-6 mutations and consistent expression of the transcription factors OCT-2, BOB.1, and PU.1 in the absence of immunoglobulins. The American Journal of Pathology, 162 (1), 243-253.

Record type: Article

Abstract

Although primary mediastinal (thymic) large B-cell lymphoma has been primarily studied, its precise phenotype, molecular characteristics, and histogenesis are still a matter of debate. The International Extranodal Lymphoma Study Group collected 137 such cases for extensive pathological review. Histologically, the lymphomatous growth was predominantly diffuse with fibrosis that induced compartmentalized cell aggregation. It consisted of large cells with varying degrees of nuclear polymorphism and clear to basophilic cytoplasm. On immunohistochemistry, the following phenotype was observed: CD45+, CD20+, CD79a+, PAX5/BSAP+, BOB.1+, Oct-2+, PU.1+, Bcl-2+, CD30+, HLA-DR+, MAL protein+/-, Bcl-6+/-, MUM1/IRF4+/-, CD10-/+, CD21-, CD15-, CD138-, CD68-, and CD3-. Immunoglobulins were negative both at immunohistochemistry and in situ hybridization. Molecular analysis, performed in 45 cases, showed novel findings. More than half of the cases displayed BCL-6 gene mutations, which usually occurred along with functioning somatic IgVH gene mutations and Bcl-6 and/or MUM1/IRF4 expression. The present study supports the concept that a sizable fraction of cases of this lymphoma are from activated germinal center or postgerminal center cells. However, it differs from other aggressive B-cell lymphomas in that it shows defective immunoglobulin production despite the expression of OCT-2, BOB.1, and PU.1 transcription factors and the lack of IgVH gene crippling mutations.

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Published date: 2003

Identifiers

Local EPrints ID: 26529
URI: http://eprints.soton.ac.uk/id/eprint/26529
ISSN: 0002-9440
PURE UUID: 82e64cb0-e1e5-45d2-b88b-730df52cf5e0

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Date deposited: 19 Apr 2006
Last modified: 22 Jul 2022 20:35

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Contributors

Author: Stefano A. Pileri
Author: Gianluca Gaidano
Author: Pier Luigi Zinzani
Author: Brunangelo Falini
Author: Philippe Gaulard
Author: Emanuele Zucca
Author: Federica Pieri
Author: Eva Berra
Author: Elena Sabattini
Author: Stefano Ascani
Author: Milena Piccioli
Author: Peter W.m. Johnson
Author: Roberto Giardini
Author: Edoardo Pescarmona
Author: Domenico Novero
Author: Pier Paolo Piccaluga
Author: Teresa Marafioti
Author: Miguel A. Alonso
Author: Franco Cavalli

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