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Updated: Jul 18, 2026

Tumor Engraftment in a Xenograft Mouse Model of Human Mantle Cell Lymphoma
Published on: March 30, 2018
Composite splenic marginal zone lymphoma and mantle cell lymphoma arising from 2 independent B-cell clones
Christine Lefebvre1, Blandine Fabre, Claire Vettier
1Inserm, E353, F-38000 Grenoble, France. clefebvre@chu-grenoble.fr
Insights
This study details the first documented case of composite lymphoma, merging mantle cell lymphoma (MCL) and splenic marginal zone lymphoma (SMZL). Genetic analysis confirmed two independent tumor clones, offering insights into complex B-cell malignancies.
Area of Science:
- Hematology
- Oncology
- Genetics
Background:
- Composite lymphomas are rare, involving two or more distinct lymphoma subtypes.
- Mantle cell lymphoma (MCL) and splenic marginal zone lymphoma (SMZL) are distinct B-cell malignancies with different clinical behaviors.
Observation:
- The study presents the first reported case of composite lymphoma involving both MCL and SMZL with circulating villous lymphocytes.
- Morphological, immunohistochemical, immunophenotyping, and genetic analyses were performed.
Findings:
- Two independent, unrelated tumor clones were confirmed: one MCL and one SMZL.
- The MCL component was CD5+, harbored t(11;14)(q13;q32) involving CCND1, and had an unmutated VH1-18 gene.
- The SMZL component was CD5-, showed t(10;14)(q24;q32) targeting NFKB2, and had an unmutated VH3-7 gene.
Implications:
- The findings suggest that MCL and SMZL arose from independent malignant transformation events in an antigen-naive B-cell population.
- This case underscores the necessity of a multidisciplinary approach and precise tissue diagnosis for complex lymphoma cases.
- The rare t(10;14)/NFKB2 translocation in SMZL warrants further investigation.
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.
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