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Review
. 2023 Sep;483(3):317-331.
doi: 10.1007/s00428-023-03633-3. Epub 2023 Sep 1.

The many faces of nodal and splenic marginal zone lymphomas. A report of the 2022 EA4HP/SH lymphoma workshop

Alberto Zamò  1 , Michiel van den Brand  2   3 , Fina Climent  4 , Laurence de Leval  5 , Stefan Dirnhofer  6 , Lorenzo Leoncini  7 , Siok-Bian Ng  8   9 , Sarah L Ondrejka  10 , Leticia Quintanilla-Martinez  11 , Lorinda Soma  12 , Andrew Wotherspoon  13
Affiliations
Review

The many faces of nodal and splenic marginal zone lymphomas. A report of the 2022 EA4HP/SH lymphoma workshop

Alberto Zamò et al. Virchows Arch. 2023 Sep.

Erratum in

Abstract

Session 3 of the lymphoma workshop of the XXI joint meeting of the European Association for Haematopathology and the Society for Hematopathology took place in Florence, Italy, on September 22, 2022. The topics of this session were splenic and nodal marginal zone lymphomas, transformation in marginal zone lymphomas, and pediatric nodal marginal zone lymphomas and their differential diagnosis as well as related entities. Forty-two cases in these categories were submitted to the workshop, including splenic lymphomas (marginal zone and diffuse red pulp lymphomas), transformed marginal zone lymphomas (splenic and nodal), nodal marginal zone lymphomas with increased TFH-cells, and pediatric nodal marginal zone lymphomas. The case review highlighted some of the principal problems in the diagnosis of marginal zone lymphomas, including the difficulties in the distinction between splenic marginal zone lymphoma, splenic diffuse red pulp lymphoma, and hairy cell leukemia variant/splenic B-cell lymphoma with prominent nucleoli which requires integration of clinical features, immunophenotype, and morphology in blood, bone marrow, and spleen; cases of marginal zone lymphoma with markedly increased TFH-cells, simulating a T-cell lymphoma, where molecular studies (clonality and mutation detection) can help to establish the final diagnosis; the criteria for transformation of marginal zone lymphomas, which are still unclear and might require the integration of morphological and molecular data; the concept of an overlapping spectrum between pediatric nodal marginal zone lymphoma and pediatric-type follicular lymphoma; and the distinction between pediatric nodal marginal zone lymphoma and "atypical" marginal zone hyperplasia, where molecular studies are mandatory to correctly classify cases.

Keywords: Marginal zone lymphoma; Marginal zone lymphoma transformation; Pediatric nodal marginal zone hyperplasia; Pediatric nodal marginal zone lymphoma; Splenic diffuse red pulp small B-cell lymphoma; Splenic marginal zone lymphoma.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
Splenic marginal zone lymphoma (SMZL) and splenic diffuse red pulp lymphoma (SDRPL); a SMZL, H&E (LYWS-1052). b SMZL, CD20 (LYWS-1052). c SDRPL, H&E (LYWS-1150). d SDRPL, CD20 (LYWS-1150)
Fig. 2
Fig. 2
Transformation of nodal marginal zone lymphoma (NMZL). In this case (LYWS-1273), some areas a still showed conventional NMZL with low-grade cytology with few admixed large cells (inset), some areas b showed an increase in large cells (inset), and some areas c showed confluent aggregates of large cells compatible with transformation (inset); the few admixed small cells were almost exclusively T-cells
Fig. 3
Fig. 3
Marginal zone lymphoma with increased T follicular helper (TFH) cells. In this case (LYWS-1051), a follicular and diffuse pattern of increased TFH cells was seen (a) with numerous CD5-positive T-cells (b) within a germinal center remnant (CD23, c). The T-cells show expression of ICOS (d), simulating a T-cell lymphoma. Clonality studies showed a B-cell clone as well as mutations associated more frequently with B-cell lymphomas and absence of clonality of T-cells as well as absence of typical mutations associated with TFH derived lymphomas
Fig. 4
Fig. 4
Pediatric nodal marginal zone lymphoma (PNMZL) and pediatric marginal zone hyperplasia (PMZH). a LYWS-1311 represents a typical PNMZL with PTGC-like changes and expanded marginal zones; b same case, CD20; c same case, CD5; d LYWS-1353 showed overlapping features between PNMZL and PTFL, including coalescing germinal centers; e same case, CD20; f same case, CD3; g LYWS-1160, PNMZH showed features of a PNMZL but was polyclonal, showed no mutations, and was associated with H. influenzae; h same case, CD20; i same case, CD5
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