Human Pathology
Volume 34, Issue 10 , Pages 1030-1034, October 2003

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

  • Laurentia Nodit, MD

      Affiliations

    • Division of Hematopathology, Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA
    • ,
  • David W Bahler, MD, PhD

      Affiliations

    • Department of Pathology, University of Utah, Salt Lake City, UT, USA
    • ,
  • Samuel A Jacobs, MD

      Affiliations

    • Division of Hematology/Oncology, Department of Medicine, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA
    • ,
  • Joseph Locker, MD, PhD

      Affiliations

    • Department of Pathology, Albert Einstein College of Medicine, Bronx, NY, USA
    • ,
  • Steven H Swerdlow, MD

      Affiliations

    • Corresponding Author InformationAddress correspondence and reprint requests to Steven H. Swerdlow, MD, Department of Pathology, Division of Hematopathology, UPMC Presbyterian—Room C606, 200 Lothrop Street, Pittsburgh, PA 15213, USA
    • Division of Hematopathology, Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA

Accepted 5 June 2003.

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.

Keywords:  mantle cell lymphoma, immunoglobulin gene mutation, B-cell lymphoma

Abbreviations:  MCL, mantel cell lymphoma, Ig, immunoglobulin, IgH, immunoglobulin heavy chain gene, FCIPS, flow cytometric immunophenotypic studies, EBV, Epstein-Barr virus, FISH, fluorescence in situ hybridization, VH, heavy chain variable gene, CBC, complete blood count, Hgb, hemoglobulin, WBC, white blood cell, CLL/SLL, B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma

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PII: S0046-8177(03)00410-6

doi:10.1053/S0046-8177(03)00410-6

Human Pathology
Volume 34, Issue 10 , Pages 1030-1034, October 2003

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