title: Splenic diffuse red pulp lymphoma has a distinct pattern of somatic mutations amongst B-cell malignancies.
abstract: 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.
authors.affiliation: a Service d'Anatomie Pathologique, Hospices Civils de Lyon , Lyon , France.|b UMR CNRS 5239 Equipe Pathologie des Cellules Lymphoïdes, Université Lyon 1 , Lyon , France.
authors.forename: Alexandra
authors.initials: A
authors.lastname: Traverse-Glehen
authors.affiliation: b UMR CNRS 5239 Equipe Pathologie des Cellules Lymphoïdes, Université Lyon 1 , Lyon , France.
authors.forename: Aurélie
authors.initials: A
authors.lastname: Verney
authors.affiliation: b UMR CNRS 5239 Equipe Pathologie des Cellules Lymphoïdes, Université Lyon 1 , Lyon , France.|c Laboratoire de Cytogénétique , Centre Hospitalier Lyon Sud , Pierre Bénite , France.
authors.forename: Sophie
authors.initials: S
authors.lastname: Gazzo
authors.affiliation: b UMR CNRS 5239 Equipe Pathologie des Cellules Lymphoïdes, Université Lyon 1 , Lyon , France.|d Laboratoire d'Hématologie cellulaire , Centre Hospitalier Lyon Sud , Pierre Bénite , France.
authors.forename: Laurent
authors.initials: L
authors.lastname: Jallades
authors.affiliation: b UMR CNRS 5239 Equipe Pathologie des Cellules Lymphoïdes, Université Lyon 1 , Lyon , France.|d Laboratoire d'Hématologie cellulaire , Centre Hospitalier Lyon Sud , Pierre Bénite , France.
authors.forename: Kaddour
authors.initials: K
authors.lastname: Chabane
authors.affiliation: b UMR CNRS 5239 Equipe Pathologie des Cellules Lymphoïdes, Université Lyon 1 , Lyon , France.|d Laboratoire d'Hématologie cellulaire , Centre Hospitalier Lyon Sud , Pierre Bénite , France.
authors.forename: Sandrine
authors.initials: S
authors.lastname: Hayette
authors.affiliation: b UMR CNRS 5239 Equipe Pathologie des Cellules Lymphoïdes, Université Lyon 1 , Lyon , France.|e Service d'Hématologie, Centre Hospitalier Lyon Sud , Pierre Bénite , France.
authors.forename: Bertrand
authors.initials: B
authors.lastname: Coiffier
authors.affiliation: b UMR CNRS 5239 Equipe Pathologie des Cellules Lymphoïdes, Université Lyon 1 , Lyon , France.|c Laboratoire de Cytogénétique , Centre Hospitalier Lyon Sud , Pierre Bénite , France.
authors.forename: Evelyne
authors.initials: E
authors.lastname: Callet-Bauchu
authors.affiliation: d Laboratoire d'Hématologie cellulaire , Centre Hospitalier Lyon Sud , Pierre Bénite , France.
authors.forename: Martine
authors.initials: M
authors.lastname: Ffrench
authors.affiliation: b UMR CNRS 5239 Equipe Pathologie des Cellules Lymphoïdes, Université Lyon 1 , Lyon , France.|d Laboratoire d'Hématologie cellulaire , Centre Hospitalier Lyon Sud , Pierre Bénite , France.
authors.forename: Pascale
authors.initials: P
authors.lastname: Felman
authors.affiliation: a Service d'Anatomie Pathologique, Hospices Civils de Lyon , Lyon , France.|b UMR CNRS 5239 Equipe Pathologie des Cellules Lymphoïdes, Université Lyon 1 , Lyon , France.
authors.forename: Françoise
authors.initials: F
authors.lastname: Berger
authors.affiliation: b UMR CNRS 5239 Equipe Pathologie des Cellules Lymphoïdes, Université Lyon 1 , Lyon , France.|d Laboratoire d'Hématologie cellulaire , Centre Hospitalier Lyon Sud , Pierre Bénite , France.
authors.forename: Lucile
authors.initials: L
authors.lastname: Baseggio
authors.affiliation: b UMR CNRS 5239 Equipe Pathologie des Cellules Lymphoïdes, Université Lyon 1 , Lyon , France.|e Service d'Hématologie, Centre Hospitalier Lyon Sud , Pierre Bénite , France.
authors.forename: Gilles
authors.initials: G
authors.lastname: Salles
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D015703:Antigens, CD / Q000235:genetics / Q000378:metabolism; D014408:Biomarkers, Tumor; D002869:Chromosome Aberrations; D004252:DNA Mutational Analysis; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D017404:In Situ Hybridization, Fluorescence; D008198:Lymph Nodes / Q000378:metabolism / Q000473:pathology; D016393:Lymphoma, B-Cell / Q000175:diagnosis / Q000235:genetics* / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D009154:Mutation; D009367:Neoplasm Staging; D012189:Retrospective Studies; D013160:Splenic Neoplasms / Q000175:diagnosis / Q000235:genetics* / Q000378:metabolism
chemical_list: D015703:Antigens, CD; D014408:Biomarkers, Tumor; C092722:CD180 protein, human
keywords: BRAF; IGHV; MYD88; NOTCH2; splenic diffuse red pulp lymphoma; splenic lymphoma