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01-05-2020 | Lymphoma | Review and Perspectives

Update on lymphoproliferative disorders of the gastrointestinal tract: disease spectrum from indolent lymphoproliferations to aggressive lymphomas

Authors: Santiago Montes-Moreno, Rebecca L. King, Ilske Oschlies, Maurilio Ponzoni, John R. Goodlad, Snjezana Dotlic, Alexandra Traverse-Glehen, German Ott, Judith A. Ferry, Maria Calaminici

Published in: Virchows Archiv | Issue 5/2020

Abstract

This paper summarizes two sessions of the workshop during the XIX meeting of the European Association for Haematopathology (EAHP) held in Edinburgh in September 2018 dedicated to lymphomas of the gastrointestinal tract. The first session focused on the clinical and pathological features of primary gastrointestinal T cell and NK-cell lymphoproliferative disorders. The distinction between precursor lesions (RCD type 2) and enteropathy-associated T cell lymphoma were stressed, including the discussion of new diagnostic markers for the identification of aberrant phenotypes. Indolent T cell lymphoproliferative disorders of the gastrointestinal tract cases showed phenotypic heterogeneity with novel molecular alterations in few cases, such as STAT3-JAK2 fusion. In addition, novel clonal markers of disease, such as AXL and JAK3 somatic variants support the neoplastic nature of NK-cell enteropathy. The session on gastrointestinal tract B cell lymphoproliferations was dedicated to B cell lymphoproliferative disorders that arise primarily in the gastrointestinal tract (i.e., duodenal-type follicular lymphoma) or preferentially involve the digestive tract, such as large B cell lymphoma with IRF4 translocation and mantle cell lymphoma (MCL), including diverse molecular subtypes (i.e., CCND3-positive MCL mimicking MALT lymphoma). Challenging cases of high-grade B cell lymphomas with complex genetic profiles demonstrated the usefulness of novel molecular diagnostic methods such as targeted NGS to identify high-risk genetic features with potential clinical impact.

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Title: Update on lymphoproliferative disorders of the gastrointestinal tract: disease spectrum from indolent lymphoproliferations to aggressive lymphomas
Authors: Santiago Montes-Moreno
Rebecca L. King
Ilske Oschlies
Maurilio Ponzoni
John R. Goodlad
Snjezana Dotlic
Alexandra Traverse-Glehen
German Ott
Judith A. Ferry
Maria Calaminici
Publication date: 01-05-2020
Publisher: Springer Berlin Heidelberg
Keywords: Lymphoma
Mantle Cell Lymphoma
Celiac Disease
Celiac Disease
Published in: Virchows Archiv / Issue 5/2020
Print ISSN: 0945-6317
Electronic ISSN: 1432-2307
DOI: https://doi.org/10.1007/s00428-019-02704-8

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Update on lymphoproliferative disorders of the gastrointestinal tract: disease spectrum from indolent lymphoproliferations to aggressive lymphomas

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