Indolent mantle cell lymphoma with nodal involvement and mutated immunoglobulin heavy chain genes.
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.
Nodit
Laurentia
L
Department of Pathology, University of Pittsburgh School of Medicine, PA, USA.
Bahler
David W
DW
Jacobs
Samuel A
SA
Locker
Joseph
J
Swerdlow
Steven H
SH
D000328:Adult; D002880:Chromosomes, Human, Pair 11; D002883:Chromosomes, Human, Pair 14; D004273:DNA, Neoplasm / Q000032:analysis; D005260:Female; D015326:Gene Rearrangement, B-Lymphocyte, Heavy Chain / Q000235:genetics*; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000235:genetics*; D007135:Immunoglobulin Variable Region / Q000235:genetics*; D007621:Karyotyping; D008198:Lymph Nodes / Q000473:pathology*; D020522:Lymphoma, Mantle-Cell / Q000235:genetics / Q000276:immunology / Q000473:pathology; D009154:Mutation; D016133:Polymerase Chain Reaction; D014178:Translocation, Genetic
D004273:DNA, Neoplasm; D007143:Immunoglobulin Heavy Chains; D007135:Immunoglobulin Variable Region