Titre
Emerging entities: high-grade/large B-cell lymphoma with 11q aberration, large B-cell lymphoma with IRF4 rearrangement, and new molecular subgroups in large B-cell lymphomas. A report of the 2022 EA4HP/SH lymphoma workshop.
Type
synthèse (review)
Institution
UNIL/CHUV/Unisanté + institutions partenaires
Périodique
Auteur(s)
Quintanilla-Martinez, L.
Auteure/Auteur
Laurent, C.
Auteure/Auteur
Soma, L.
Auteure/Auteur
Ng, S.B.
Auteure/Auteur
Climent, F.
Auteure/Auteur
Ondrejka, S.L.
Auteure/Auteur
Zamo, A.
Auteure/Auteur
Wotherspoon, A.
Auteure/Auteur
de Leval, L.
Auteure/Auteur
Dirnhofer, S.
Auteure/Auteur
Leoncini, L.
Auteure/Auteur
Liens vers les personnes
Liens vers les unités
ISSN
1432-2307
Statut éditorial
Publié
Date de publication
2023-09
Volume
483
Numéro
3
Première page
281
Dernière page/numéro d’article
298
Peer-reviewed
Oui
Langue
anglais
Notes
Publication types: Journal Article ; Review
Publication Status: ppublish
Publication Status: ppublish
Résumé
Emerging entities and molecular subgroups in large B-cell lymphomas (LBCLs) were discussed during the 2022 European Association for Haematopathology/Society for Hematopathology workshop in Florence, Italy. This session focused on newly recognized diseases and their diagnostic challenges. High-grade/large B-cell lymphoma with 11q aberration (HG/LBCL-11q) is defined by chromosome 11q-gains and telomeric loss. FISH analysis is recommended for the diagnosis. HG/LBCL-11q can occur in the setting of immunodeficiency, including ataxia-telangiectasia, and predominates in children. The morphological spectrum of these cases is broader than previously thought with often Burkitt-like morphology and coarse apoptotic bodies. It has a Burkitt-like immunophenotype (CD10+, BCL6+, BCL2-) but MYC expression is weak or negative, lacks MYC rearrangement, and is in contrast to Burkitt lymphoma 50% of the cases express LMO2. LBCL with IRF4 rearrangement (LBCL-IRF4) occurs mainly in the pediatric population but also in adults. LBCL-IRF4 has an excellent prognosis, with distinguishing molecular findings. IRF4 rearrangements, although characteristic of this entity, are not specific and can be found in association with other chromosomal translocations in other large B-cell lymphomas. Other molecular subgroups discussed included primary bone diffuse large B-cell lymphoma (PB-DLBCL), which has distinctive clinical presentation and molecular findings, and B-acute lymphoblastic leukemia (B-ALL) with IGH::MYC translocation recently segregated from Burkitt lymphoma with TdT expression. This latter disorder has molecular features of precursor B-cells, often tetrasomy 1q and recurrent NRAS and KRAS mutations. In this report, novel findings, recommendations for diagnosis, open questions, and diagnostic challenges raised by the cases submitted to the workshop will be discussed.
PID Serval
serval:BIB_8A55266746F3
PMID
Open Access
Oui
Date de création
2023-08-10T11:06:40.050Z
Date de création dans IRIS
2025-05-21T00:39:48Z
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Nom
s00428-023-03590-x.pdf
Version du manuscrit
published
Licence
https://creativecommons.org/licenses/by/4.0
Taille
3.95 MB
Format
Adobe PDF
PID Serval
serval:BIB_8A55266746F3.P001
URN
urn:nbn:ch:serval-BIB_8A55266746F36
Somme de contrôle
(MD5):a2f6d0b3970c517c0a4441c4d9c4b11c