Transformations of marginal zone lymphomas and lymphoplasmacytic lymphomas: Report from the 2021 SH/eAHP workshop Journal Article


Authors: Cook, J. R.; Amador, C.; Czader, M.; Duffield, A.; Goodlad, J.; Ott, G.; Xiao, W.; Dave, S.; Thakkar, D.; Thacker, E.; Dogan, A.; Wasik, M.; Nejati, R.
Article Title: Transformations of marginal zone lymphomas and lymphoplasmacytic lymphomas: Report from the 2021 SH/eAHP workshop
Abstract: Objectives: To summarize the conclusions of the 2021 Society for Hematopathology/ European Association for Haematopathology workshop regarding transformations of marginal zone lymphoma (MZL) and lymphoplasmacytic lymphoma (LPL). Methods: Nineteen cases were submitted to this portion of the workshop. Additional studies were performed in cases with sufficient material. Results: Cases included splenic MZL (n = 4), splenic diffuse red pulp small B-cell lymphoma (n = 2), nodal MZL (n = 4), extranodal MZL (n = 1), and LPL (n = 8). The most common transformation was to diffuse large B-cell lymphoma (DLBCL), but others included classic Hodgkin lymphoma, high-grade B-cell lymphomas with MYC and BCL6 rearrangements, plasmablastic lymphoma, and plasma cell leukemia. Two splenic MZLs with transformation to DLBCL contained t(14;19)(q32;q13.3) IGH::BCL3 rearrangements in both samples. Paired sequencing studies in 5 MZLs with transformation to clonally related DLBCL identified a variety of mutations and gene fusions at the time of transformation, including CARD11, IGH::MYC, NOTCH2, P2RY8, TBLX1X1, and IGH::CD274. Conclusions: Marginal zone lymphoma and LPL may undergo a variety of transformation events, most commonly to DLBCL, which is usually, although not always, directly clonally related to the underlying low-grade lymphoma. Multiparameter analysis including broad-based sequencing studies can assist in the diagnosis and classification of these uncommon cases. © the author(s) 2023. published by oxford University press on behalf of american society for clinical pathology. all rights reserved.
Keywords: marginal zone lymphoma; transformation; transdifferentiation; lymphoplasmacytic lymphoma
Journal Title: American Journal of Clinical Pathology
Volume: 159
Issue: 6
ISSN: 0002-9173
Publisher: Oxford University Press  
Date Published: 2023-06-01
Start Page: 614
End Page: 625
Language: English
DOI: 10.1093/ajcp/aqad034
PUBMED: 37186259
PROVIDER: scopus
DOI/URL:
Notes: Review -- Source: Scopus
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  1. Ahmet Dogan
    454 Dogan
  2. Wenbin Xiao
    108 Xiao