Indolent mantle cell lymphoma with nodal involvement and mutated immunoglobulin heavy chain genes

Hum Pathol. 2003 Oct;34(10):1030-4. doi: 10.1053/s0046-8177(03)00410-6.

Abstract

Mantle cell lymphoma (MCL) is typically considered an aggressive but incurable neoplasm composed of cyclin D1+ monoclonal B-cells with a t(11;14)(q13;q32) and usually unmutated immunoglobulin (Ig) genes. Although it has been suggested that a more indolent leukemic disorder exists with the same phenotype and genotype but with mutated Ig genes, others have considered these cases to be variants of chronic lymphocytic leukemia. We present a case of an indolent MCL that was documented with cyclin D1 expression in a lymph node biopsy performed more than 12 years ago. The patient has peripheral blood involvement with a lymphocyte count in the reference range, variable thrombocytopenia, and minimal adenopathy but is otherwise well, never having received any antineoplastic therapy. Study of peripheral blood samples from 2002 revealed a CD5-variable B-cell monoclonal proliferation with a t(11;14)(q13;q32) plus other karyotypic abnormalities, positive fluorescence in situ hybridization studies for the CCND1/IgH translocation, and clonal Ig gene rearrangement with mutated Ig genes (95.7% homology to VH 4-31). The subtle but diagnostic lymph node biopsy in this case helps to further support that an indolent t(11;14) monoclonal lymphocytosis with mutated Ig genes can represent an MCL variant rather than chronic lymphocytic leukemia.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Chromosomes, Human, Pair 11
  • Chromosomes, Human, Pair 14
  • DNA, Neoplasm / analysis
  • Female
  • Gene Rearrangement, B-Lymphocyte, Heavy Chain / genetics*
  • Humans
  • Immunoglobulin Heavy Chains / genetics*
  • Immunoglobulin Variable Region / genetics*
  • Karyotyping
  • Lymph Nodes / pathology*
  • Lymphoma, Mantle-Cell* / genetics
  • Lymphoma, Mantle-Cell* / immunology
  • Lymphoma, Mantle-Cell* / pathology
  • Mutation*
  • Polymerase Chain Reaction
  • Translocation, Genetic

Substances

  • DNA, Neoplasm
  • Immunoglobulin Heavy Chains
  • Immunoglobulin Variable Region