title: Composite splenic marginal zone lymphoma and mantle cell lymphoma arising from 2 independent B-cell clones.
abstract: We report the first case of composite lymphoma involving both mantle cell lymphoma (MCL) and splenic marginal zone lymphoma (SMZL) with circulating villous lymphocytes. Morphological, immunohistochemical, immunophenotyping, as well as detailed genetic studies (fluorescence in situ hybridization, IGVH gene sequencing), were performed and confirmed the existence of 2 independent, unrelated tumor clones. The MCL component expressed IgMD lambda, was CD5+, harbored a t(11;14)(q13;q32) involving CCND1, and showed an unmutated VH1-18 gene rearrangement. The SMZL component expressed IgMD kappa, was CD5-, showed a t(10;14)(q24;q32) and an unmutated VH3-7 gene rearrangement. Interestingly, this t(10;14) targeted the NFKB2 gene. Only a single other case of SMZL with t(10;14)/NFKB2 has been reported. Taken together, these data indicate that the MCL and SMZL arose as a consequence of independent malignant transformation events within an antigen-naive B-cell population. This case highlights the importance of a multidisciplinary approach and tissue diagnosis in these complex situations.
authors.affiliation: Inserm, E353, F-38000 Grenoble, France. clefebvre@chu-grenoble.fr
authors.forename: Christine
authors.initials: C
authors.lastname: Lefebvre
authors.forename: Blandine
authors.initials: B
authors.lastname: Fabre
authors.forename: Claire
authors.initials: C
authors.lastname: Vettier
authors.forename: Laetitia
authors.initials: L
authors.lastname: Rabin
authors.forename: Anne
authors.initials: A
authors.lastname: Florin
authors.forename: Jin
authors.initials: J
authors.lastname: Wang
authors.forename: Rémy
authors.initials: R
authors.lastname: Gressin
authors.forename: Marie-Christine
authors.initials: MC
authors.lastname: Jacob
authors.forename: Mary
authors.initials: M
authors.lastname: Callanan
authors.forename: Dominique
authors.initials: D
authors.lastname: Leroux
mesh_terms: D000368:Aged; D056741:Cyclin D; D016213:Cyclins / Q000235:genetics; D006801:Humans; D007150:Immunohistochemistry; D016393:Lymphoma, B-Cell / Q000737:chemistry / Q000473:pathology*; D020522:Lymphoma, Mantle-Cell / Q000737:chemistry / Q000473:pathology*; D008228:Lymphoma, Non-Hodgkin / Q000737:chemistry / Q000473:pathology*; D008297:Male; D052003:NF-kappa B p52 Subunit / Q000235:genetics; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D013160:Splenic Neoplasms / Q000737:chemistry / Q000473:pathology*
chemical_list: D056741:Cyclin D; D016213:Cyclins; D052003:NF-kappa B p52 Subunit; C498206:NFKB2 protein, human