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Diffuse large B-cell lymphoma variants: an update.

Narittee Sukswai1, Kirill Lyapichev2, Joseph D Khoury2

  • 1Department of Pathology, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand; Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.

Pathology
|November 19, 2019
PubMed
Summary

Diffuse large B-cell lymphoma (DLBCL) encompasses numerous variants beyond the standard classification. This review details specific DLBCL subtypes, highlighting unique features and suggesting future WHO classification changes for better diagnosis and treatment.

Keywords:
Diffuse large B-cell lymphomaWorld Health Organization classificationvariants

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Area of Science:

  • Hematology
  • Oncology
  • Pathology

Background:

  • Diffuse large B-cell lymphoma (DLBCL) is the most prevalent form of lymphoma globally, accounting for about one-third of all lymphoma cases.
  • While most DLBCL cases are classified as 'not otherwise specified' (NOS), approximately 20% are recognized as distinct variants.
  • These variants are defined by unique morphological, immunophenotypic, biological, or clinical characteristics.

Purpose of the Study:

  • To review and discuss the recognized specific variants of DLBCL.
  • To highlight the diagnostic criteria and associated features of these variants.
  • To propose the inclusion of primary testicular DLBCL as a specific variant in future WHO classifications.

Main Methods:

  • Review of current literature and the World Health Organization (WHO) classification of lymphomas.
  • Discussion of 13 specific DLBCL variants based on established diagnostic criteria.
  • Analysis of unique biological features of primary testicular DLBCL.

Main Results:

  • Detailed discussion of variants including T-cell/histiocyte-rich large B-cell lymphoma, ALK-positive large B-cell lymphoma, plasmablastic lymphoma, and others.
  • Identification of primary testicular DLBCL as a distinct entity with unique biological features.
  • Recognition that EBV-positive DLBCL and EBV-positive mucocutaneous ulcer are discussed in a separate review.

Conclusions:

  • The current WHO classification recognizes specific variants of DLBCL based on distinct features.
  • Primary testicular DLBCL exhibits unique characteristics warranting its recognition as a specific variant.
  • Future WHO classifications should consider incorporating primary testicular DLBCL for improved patient management.