cyclin-d1 and Abnormal-Karyotype

We report herein a case of blastoid variant mantle cell lymphoma (MCL) with both aberrant phenotype and unusual genetics. Unexpectedly, lymphoma cells were CD5(-) and CD10(+). Standard karyotype and FISH techniques showed that tumor cells carried two distinct translocations which had not been reported together in a same tumor. The first translocation juxtaposed the immunoglobulin lambda light chain locus with CCND1 locus, leading to Cyclin D1 overexpression. The second translocation revealed MYC rearrangement with a non-immunoglobulin gene partner located on the short arm of chromosome 4. The interpretation of the case on tissue sections alone could have been challenging. Indeed, the lack of CD5 and expression of CD10 associated with MYC rearrangement detected on interphasic nuclei could support the diagnosis of diffuse large B-cell lymphoma or Burkitt lymphoma. This distinction is also especially important as these lymphoma subtypes require specific treatment.

Topics: Abnormal Karyotype; Aged, 80 and over; Chromosomes, Human, Pair 11; Chromosomes, Human, Pair 22; Cyclin D1; Female; Genes, myc; Humans; Immunohistochemistry; In Situ Hybridization, Fluorescence; Lymphoma, Mantle-Cell; Translocation, Genetic

Topics: Abnormal Karyotype; Adenine; Aged; Antineoplastic Combined Chemotherapy Protocols; Bendamustine Hydrochloride; Biomarkers, Tumor; Bortezomib; Chromosomes, Human, Pair 11; Chromosomes, Human, Pair 14; Chromosomes, Human, Pair 8; Cyclin D1; Cytarabine; Drug Resistance, Neoplasm; Fatal Outcome; Gene Duplication; Genes, myc; Humans; Lymphoma, Mantle-Cell; Male; Oncogene Proteins, Fusion; Piperidines; Pyrazoles; Pyrimidines; Recurrence; Rituximab; Translocation, Genetic