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

James R. Cook, Catalina Amador, Magdalena Czader, Amy Duffield, John Goodlad, German Ott, Wenbin Xiao, Sandeep Dave, Devang Thakkar, Elizabeth Thacker, Ahmet Dogan, Mariusz Wasik, Reza Nejati

Research output: Contribution to journalArticlepeer-review

3 Scopus citations

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.

Original languageEnglish
Pages (from-to)614-625
Number of pages12
JournalAmerican Journal of Clinical Pathology
Volume159
Issue number6
Early online dateMar 26 2023
DOIs
StatePublished - Jun 1 2023

Keywords

  • Lymphoplasmacytic lymphoma
  • Marginal zone lymphoma
  • Transdifferentiation
  • Transformation

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