Summary
Diffuse large B-cell lymphoma (DLBCL) is the most common type of lymphoma, representing
approximately one-third of all cases worldwide. In the World Health Organization (WHO)
classification of lymphomas, most cases of DLBCL are designated as not otherwise specified
(NOS). About 20% of cases, however, are designated as specific variants of DLBCL.
These variants, 13 in total, are specified on the basis of distinctive morphological
or immunophenotypic findings or distinctive biological or clinical issues associated
with their diagnoses. In this review we discuss the following variants: T-cell/histiocyte-rich
large B-cell lymphoma; ALK-positive large B-cell lymphoma; plasmablastic lymphoma;
intravascular large B-cell lymphoma; large B-cell lymphoma with IRF4 rearrangement; primary mediastinal large B-cell lymphoma; primary cutaneous diffuse
large B-cell lymphoma, leg type; primary diffuse large B-cell lymphoma of the central
nervous system; diffuse large B-cell lymphoma associated with chronic inflammation;
lymphomatoid granulomatosis; primary effusion lymphoma; and HHV8-positive diffuse
large B-cell lymphoma, NOS. Two additional variants recognised in the WHO classification,
EBV-positive diffuse large B-cell lymphoma and EBV-positive mucocutaneous ulcer are
discussed elsewhere in another review within this issue of Pathology. Although not recognised as a specific variant in the current WHO classification,
primary testicular diffuse large B-cell lymphoma also has unique biological features
and requires some modification of the standard treatment approach for patients with
DLBCL. Therefore, we suggest that primary testicular diffuse large B-cell lymphoma
also should be recognised as a specific variant of DLBCL in a future version of the
WHO classification.
Key words
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Article Info
Publication History
Published online: November 15, 2019
Accepted:
August 28,
2019
Received in revised form:
August 26,
2019
Received:
August 11,
2019
Identification
Copyright
© 2019 Royal College of Pathologists of Australasia. Published by Elsevier B.V. All rights reserved.


