Clinical and Pathological Features of Double-Hit and Triple-Hit High-Grade B-Cell Lymphomas: A Retrospective Study from Three Portuguese Tertiary Centers

Authors

  • Rui Almeida Department of Pathology, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal
  • Carlos Abrantes Department of Anatomical Pathology, Hospital of the University of Coimbra (CHUC), Portugal
  • Davide Gigliano Department of Pathology, Portuguese Oncology Institute of Porto, Porto, Portugal
  • Rui Caetano Oliveira Department of Pathology, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal
  • Paulo Teixeira Department of Pathology, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal
  • Marta Viegas Molecular Pathology Laboratory, Instituto Português de Oncologia de Coimbra de Francisco Gentil, 3000-651 Coimbra, Portugal
  • Ângelo Rodrigues Department of Pathology, Portuguese Oncology Institute of Porto, Porto, Portugal
  • Maria José Julião Department of Pathology, Coimbra Hospital and University Centre, CHUC, EPE, 3000-075, Coimbra, Portugal

DOI:

https://doi.org/10.18502/ijhoscr.v16i2.9202

Keywords:

High-grade; B-cell; Lymphoma; Cytogenetics; Immunohistochemistry; Double-hit; Triple-hit

Abstract

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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Published

2022-04-17

Issue

Volume (16) issue (2)

Section

Articles