Clinical and Pathological Features of Double-Hit and Triple-Hit High-Grade B-Cell Lymphomas: A Retrospective Study from Three Portuguese Tertiary Centers
Background: High-grade B-cell lymphoma (HGBL) with rearrangements of MYC and BCL2 and/or BCL6, called double and triple-hit lymphomas (DTH-HGBL), are lymphoid malignancies with inferior outcomes when treated with standard chemotherapy. The identification of DTH-HGBL cases is challenging, cons...
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| Format: | Article |
| Language: | English |
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Tehran University of Medical Sciences
2022-04-01
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| Series: | International Journal of Hematology-Oncology and Stem Cell Research |
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| Online Access: | https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/1351 |
| _version_ | 1797709960606384128 |
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| author | Rui Almeida Carlos Abrantes Davide Gigliano Rui Caetano Oliveira Paulo Teixeira Marta Viegas Ângelo Rodrigues Maria José Julião |
| author_facet | Rui Almeida Carlos Abrantes Davide Gigliano Rui Caetano Oliveira Paulo Teixeira Marta Viegas Ângelo Rodrigues Maria José Julião |
| author_sort | Rui Almeida |
| collection | DOAJ |
| description |
Background: High-grade B-cell lymphoma (HGBL) with rearrangements of MYC and BCL2 and/or BCL6, called double and triple-hit lymphomas (DTH-HGBL), are lymphoid malignancies with inferior outcomes when treated with standard chemotherapy. The identification of DTH-HGBL cases is challenging, considering their variable clinical, morphologic, and immunohistochemical features.
Materials and Methods: Retrospective revision of medical data of patients diagnosed with DTH-HGBL confirmed by FISH, between January 2010 and January 2020, in three Tertiary Portuguese Hospitals (Coimbra Hospital and University Center, Portuguese Oncology Institute – Coimbra and Portuguese Oncology Institute – Porto). Pathological features, morphology, and immunohistochemical profile were evaluated by at least two experienced pathologists in hematopoietic and lymphoid neoplasms.
Results: The cohort included 24 patients: 33.3% triple-hit, 58.3%, MYC/BCL2 double-hit and 8.3% MYC/BCL6 double-hit. There was no gender predominance, with a median age of 62.5±14.3y, 33.3% were diagnosed as nodal disease and 66.7% as extranodal.
Morphologic features of DLBCL were present in 50% of cases, morphological features of both DLBCL and Burkitt lymphoma (DLBCL/BL) in 45.8% and 4.2% of blastoid morphology.
Immunohistochemical evaluation, regarding the Hans algorithm, revealed a Germinal center (GC)/GC-like subtype in 83.3% of cases and a non-GC/non-GC-like subtype in 16.7%. MYC was positive in 42.9% and the median proliferative index was 80±12.4%.
Conclusion: DTH-HGBL has a very broad range of features. We consider that a cost-effective approach would be to perform cytogenetic analysis in DLBCL and DLBCL/BL cases with GC/GC-like subtype. MYC and BCL2 immunohistochemistry can be useful to identify patients who may benefit from more aggressive therapies, but not as tools for case selection for FISH.
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| first_indexed | 2024-03-12T06:45:24Z |
| format | Article |
| id | doaj.art-9a3064225b0646b8b8509a22f9023391 |
| institution | Directory Open Access Journal |
| issn | 2008-2207 |
| language | English |
| last_indexed | 2024-03-12T06:45:24Z |
| publishDate | 2022-04-01 |
| publisher | Tehran University of Medical Sciences |
| record_format | Article |
| series | International Journal of Hematology-Oncology and Stem Cell Research |
| spelling | doaj.art-9a3064225b0646b8b8509a22f90233912023-09-03T00:38:46ZengTehran University of Medical SciencesInternational Journal of Hematology-Oncology and Stem Cell Research2008-22072022-04-01162Clinical and Pathological Features of Double-Hit and Triple-Hit High-Grade B-Cell Lymphomas: A Retrospective Study from Three Portuguese Tertiary CentersRui Almeida0Carlos Abrantes1Davide Gigliano2Rui Caetano Oliveira3Paulo Teixeira4Marta Viegas5Ângelo Rodrigues6Maria José Julião7Department of Pathology, Centro Hospitalar e Universitário de Coimbra, Coimbra, PortugalDepartment of Anatomical Pathology, Hospital of the University of Coimbra (CHUC), PortugalDepartment of Pathology, Portuguese Oncology Institute of Porto, Porto, PortugalDepartment of Pathology, Centro Hospitalar e Universitário de Coimbra, Coimbra, PortugalDepartment of Pathology, Centro Hospitalar e Universitário de Coimbra, Coimbra, PortugalMolecular Pathology Laboratory, Instituto Português de Oncologia de Coimbra de Francisco Gentil, 3000-651 Coimbra, PortugalDepartment of Pathology, Portuguese Oncology Institute of Porto, Porto, PortugalDepartment of Pathology, Coimbra Hospital and University Centre, CHUC, EPE, 3000-075, Coimbra, Portugal Background: High-grade B-cell lymphoma (HGBL) with rearrangements of MYC and BCL2 and/or BCL6, called double and triple-hit lymphomas (DTH-HGBL), are lymphoid malignancies with inferior outcomes when treated with standard chemotherapy. The identification of DTH-HGBL cases is challenging, considering their variable clinical, morphologic, and immunohistochemical features. Materials and Methods: Retrospective revision of medical data of patients diagnosed with DTH-HGBL confirmed by FISH, between January 2010 and January 2020, in three Tertiary Portuguese Hospitals (Coimbra Hospital and University Center, Portuguese Oncology Institute – Coimbra and Portuguese Oncology Institute – Porto). Pathological features, morphology, and immunohistochemical profile were evaluated by at least two experienced pathologists in hematopoietic and lymphoid neoplasms. Results: The cohort included 24 patients: 33.3% triple-hit, 58.3%, MYC/BCL2 double-hit and 8.3% MYC/BCL6 double-hit. There was no gender predominance, with a median age of 62.5±14.3y, 33.3% were diagnosed as nodal disease and 66.7% as extranodal. Morphologic features of DLBCL were present in 50% of cases, morphological features of both DLBCL and Burkitt lymphoma (DLBCL/BL) in 45.8% and 4.2% of blastoid morphology. Immunohistochemical evaluation, regarding the Hans algorithm, revealed a Germinal center (GC)/GC-like subtype in 83.3% of cases and a non-GC/non-GC-like subtype in 16.7%. MYC was positive in 42.9% and the median proliferative index was 80±12.4%. Conclusion: DTH-HGBL has a very broad range of features. We consider that a cost-effective approach would be to perform cytogenetic analysis in DLBCL and DLBCL/BL cases with GC/GC-like subtype. MYC and BCL2 immunohistochemistry can be useful to identify patients who may benefit from more aggressive therapies, but not as tools for case selection for FISH. https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/1351High-grade; B-cell; Lymphoma; Cytogenetics; Immunohistochemistry; Double-hit; Triple-hit |
| spellingShingle | Rui Almeida Carlos Abrantes Davide Gigliano Rui Caetano Oliveira Paulo Teixeira Marta Viegas Ângelo Rodrigues Maria José Julião Clinical and Pathological Features of Double-Hit and Triple-Hit High-Grade B-Cell Lymphomas: A Retrospective Study from Three Portuguese Tertiary Centers International Journal of Hematology-Oncology and Stem Cell Research High-grade; B-cell; Lymphoma; Cytogenetics; Immunohistochemistry; Double-hit; Triple-hit |
| title | Clinical and Pathological Features of Double-Hit and Triple-Hit High-Grade B-Cell Lymphomas: A Retrospective Study from Three Portuguese Tertiary Centers |
| title_full | Clinical and Pathological Features of Double-Hit and Triple-Hit High-Grade B-Cell Lymphomas: A Retrospective Study from Three Portuguese Tertiary Centers |
| title_fullStr | Clinical and Pathological Features of Double-Hit and Triple-Hit High-Grade B-Cell Lymphomas: A Retrospective Study from Three Portuguese Tertiary Centers |
| title_full_unstemmed | Clinical and Pathological Features of Double-Hit and Triple-Hit High-Grade B-Cell Lymphomas: A Retrospective Study from Three Portuguese Tertiary Centers |
| title_short | Clinical and Pathological Features of Double-Hit and Triple-Hit High-Grade B-Cell Lymphomas: A Retrospective Study from Three Portuguese Tertiary Centers |
| title_sort | clinical and pathological features of double hit and triple hit high grade b cell lymphomas a retrospective study from three portuguese tertiary centers |
| topic | High-grade; B-cell; Lymphoma; Cytogenetics; Immunohistochemistry; Double-hit; Triple-hit |
| url | https://ijhoscr.tums.ac.ir/index.php/ijhoscr/article/view/1351 |
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