Splenic diffuse red pulp lymphoma has a distinct pattern of somatic mutations amongst B-cell malignancies

Splenic Diffuse Red Pulp Lymphoma (SDRPL) has been recently introduced as a provisional entity but differential diagnosis with other splenic lymphomas is needed to be clarified since the therapeutic approaches are distinct. Recently described recurrent mutations or CD180 expression appear useful for...

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Bibliographic Details
Published in:Leukemia & lymphoma 2017-03, Vol.58 (3), p.666-675
Main Authors: Traverse-Glehen, Alexandra, Verney, Aurélie, Gazzo, Sophie, Jallades, Laurent, Chabane, Kaddour, Hayette, Sandrine, Coiffier, Bertrand, Callet-Bauchu, Evelyne, Ffrench, Martine, Felman, Pascale, Berger, Françoise, Baseggio, Lucile, Salles, Gilles
Format: Article
Language:English
Subjects:
Aged
Aged, 80 and over
Antigens, CD - genetics
Antigens, CD - metabolism
Biomarkers, Tumor
BRAF
Cancer
Chromosome Aberrations
DNA Mutational Analysis
Female
Genetic Predisposition to Disease
Humans
IGHV
Immunohistochemistry
Immunophenotyping
In Situ Hybridization, Fluorescence
Life Sciences
Lymph Nodes - metabolism
Lymph Nodes - pathology
Lymphoma, B-Cell - diagnosis
Lymphoma, B-Cell - genetics
Lymphoma, B-Cell - metabolism
Male
Middle Aged
Mutation
MYD88
Neoplasm Staging
NOTCH2
Retrospective Studies
splenic diffuse red pulp lymphoma
splenic lymphoma
Splenic Neoplasms - diagnosis
Splenic Neoplasms - genetics
Splenic Neoplasms - metabolism
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Description
Summary:Splenic Diffuse Red Pulp Lymphoma (SDRPL) has been recently introduced as a provisional entity but differential diagnosis with other splenic lymphomas is needed to be clarified since the therapeutic approaches are distinct. Recently described recurrent mutations or CD180 expression appear useful for differential diagnosis. We completed our previous description in a larger cohort including 53 patients selected on the presence of characteristic villous cells in peripheral blood (PB) and a specific immunophenotype. Immunoglobulin heavy variable (IGHV), BRAF, MYD88, and NOTCH2 mutations were determined and CD180 and BRAF expressions were assessed. Most cases (79%) were IGHV mutated with an overrepresentation of IGHV3-23 (19%) and IGHV4-34 (21%). MYD88 L265P and NOTCH2 mutations were observed in one case each, whereas no BRAF V600E mutation or expression was found. All cases demonstrated a high CD180 expression. Those results strengthen the concept that SDRPL does emerge as a new lymphoma entity distinct from the other splenic lymphomas with circulating lymphocytes.
ISSN:1042-8194
1029-2403
DOI:10.1080/10428194.2016.1196813