Evolution in the definition and diagnosis of the Hodgkin lymphomas and related entities

Thomas A. Tousseyn, Rebecca L. King, Falko Fend, Andrew L. Feldman, Pierre Brousset, Elaine S. Jaffe

Research output: Contribution to journalReview articlepeer-review

Abstract

Hodgkin lymphoma was the first of the lymphomas to be recognized as a specific disease entity. However, recent studies have highlighted the heterogeneity of the diseases associated with this eponym warranting clarification and refinement of diagnostic terminology. While classic Hodgkin lymphoma (CHL) remains an essentially unchanged diagnostic entity in the 2022 International Consensus Classification of Mature Lymphoid Neoplasms (2022 ICC), nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is now renamed nodular lymphocyte predominant B cell lymphoma (NLPBL) in recognition of the distinct pathologic, biologic, and clinical differences. Fan patterns A, B, and C (sharing the presence of evident follicular structures, and retention of a B cell rich background) will be combined in “typical” or grade 1, while the other “variant” patterns, D, E, and F, are considered grade 2. T-cell/histiocyte-rich large B cell lymphoma (THRBCL) is considered part of the “variant” NLPHL continuum. The entity previously known as “B cell lymphoma, unclassifiable (BCLU), with features intermediate between diffuse large B cell lymphoma (DLBCL) and CHL” has been renamed “mediastinal gray zone lymphoma” (MGZL) in recognition of the importance of the thymic niche in the biology of this tumor. The diagnostic criteria for MGZL have been refined and require both a high tumor cell density and a strongly preserved B cell program. This article will describe updates on CHL, NLPBL, and MGZL in the recently published 2022 ICC and provide some useful differential diagnostic clues in cases with atypical morphology or immunophenotype.

Original languageEnglish (US)
JournalVirchows Archiv
DOIs
StateAccepted/In press - 2022

Keywords

  • 2022 ICC
  • Classic Hodgkin lymphoma
  • Mediastinal gray zone lymphoma
  • Nodular lymphocyte predominant B cell lymphoma
  • T cell/histiocyte-rich large B cell lymphoma

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Molecular Biology
  • Cell Biology

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