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Review
. 2011:2011:506-14.
doi: 10.1182/asheducation-2011.1.506.

Aggressive B-cell lymphomas: a review of new and old entities in the WHO classification

Elaine S Jaffe  1 , Stefania Pittaluga
Affiliations
Review

Aggressive B-cell lymphomas: a review of new and old entities in the WHO classification

Elaine S Jaffe et al. Hematology Am Soc Hematol Educ Program. 2011.

Abstract

Aggressive B-cell lymphomas are clinically and pathologically diverse and reflect multiple pathways of transformation. The 2008 World Health Organization (WHO) classification reflects this complexity with the addition of several new entities and variants. Whereas MYC translocations have long been associated with Burkitt lymphoma (BL), deregulation of MYC has been shown to occur in other aggressive B-cell lymphomas, most often as a secondary event. Lymphomas with translocations of both MYC and BCL2 are highly aggressive tumors, with a high failure rate with most treatment protocols. These "double-hit" lymphomas are now separately delineated in the WHO classification as B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma (DLBCL) and BL. A MYC translocation is also found uncommonly in DLBCL, but the clinical consequences of this in the absence of a double hit are not yet fully delineated. Most recently, MYC translocations have been identified as a common secondary event in plasma cell neoplasms, seen in approximately 50% of plasmablastic lymphoma. Another area that has received recent attention is the spectrum of EBV-driven B-cell proliferations in patients without iatrogenic or congenital immunosuppression; most of these occur in patients of advanced age and include the EBV-positive large B-cell lymphomas of the elderly.

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Conflict of interest statement

Conflict-of-interest disclosures: The authors declare no competing financial interests. Off-label drug use: None disclosed.

Figures

Figure 1.
Figure 1.. EBV-positive DLBCL of the elderly.
Large lymphoid cells, some with prominent inclusion-like nucleoli (inset) are seen in a background of lymphocytes and histiocytes. Large lymphoid cells are EBV positive with EBER in situ hybridization (not shown).
Figure 2.
Figure 2.. BL with immunohistochemistry for MYC protein.
(Left) Section showing BL associated with an epithelioid granulomatous reaction. (Right) Same case stained for MYC protein; tumor cells show strong nuclear positivity, whereas epithelioid histiocytes are negative for MYC. Strong nuclear staining is typical of BL, but may be seen in some cases of DLBCL with a high proliferative rate.
Figure 3.
Figure 3.. Differential diagnosis of BL, B-UNC/BL/DLBCL, and DLBCL of the GCB type.
B-UNC/BL/DLBCL is generally associated with a complex karyotype containing both MYC and BCL2 translocations. BCL2 translocations are found in approximately 30% of the GCB type of DLBCL, but MYC translocation should be absent, and if a dual translocation is found, the case should be classified as B-UNC/BL/DLBCL. Morphologically, BL is composed of medium-sized cells, with minimal nuclear variation. Cell size is largest in DLBCL, and B-UNC/BL/DLBCL is generally composed of cells of intermediate to large size, with greater variability than BL.
See this image and copyright information in PMC

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