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

Human Pathology
|December 1, 2006
PubMed

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.

Related Concept Videos

Cells of the Adaptive Immune Response01:23

Cells of the Adaptive Immune Response

The T and B lymphocytes of the adaptive immune system develop from common lymphoid progenitor cells in the bone marrow. These progenitors give rise to precursors that eventually develop into both T and B lymphocytes. As these precursors mature, they gain the ability to detect and respond to foreign antigens in the body, a process known as immunocompetence. Additionally, these precursors acquire self-tolerance, a process that ensures they do not react to self-antigens. This intricate system...
T Cell Activation and Clonal Selection01:22

T Cell Activation and Clonal Selection

T cells are integral to our adaptive immune system, recognizing and effectively responding to foreign antigens. T cell activation and clonal selection are pivotal in orchestrating this immune response. This article elucidates these mechanisms, detailing the roles of cluster of differentiation (CD) markers, major histocompatibility complex (MHC) molecules, costimulatory signals, and the process of clonal selection.
Naive T cells that have not yet encountered an antigen express two primary CD...
Secondary Lymphoid Organs01:15

Secondary Lymphoid Organs

Secondary organs, including lymph nodes, the spleen, and mucosa-associated lymphoid tissue (MALT), work harmoniously to protect us from disease and infection.
The spleen is a vital organ in the lymphatic system, nestled in the upper left side of the abdomen. It is composed of two primary regions: the red pulp and the white pulp, each having distinct functions. The red pulp performs a significant role in blood filtration. It efficiently purges the blood of old or damaged red blood cells and...