Composite splenic marginal zone lymphoma and mantle cell lymphoma arising from 2 independent B-cell clones.
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.
Lefebvre
Christine
C
Inserm, E353, F-38000 Grenoble, France. clefebvre@chu-grenoble.fr
Fabre
Blandine
B
Vettier
Claire
C
Rabin
Laetitia
L
Florin
Anne
A
Wang
Jin
J
Gressin
Rémy
R
Jacob
Marie-Christine
MC
Callanan
Mary
M
Leroux
Dominique
D
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*
D056741:Cyclin D; D016213:Cyclins; D052003:NF-kappa B p52 Subunit; C498206:NFKB2 protein, human