Dual Rearrangement of Immunoglobulin and T-cell Receptor Genes in a Case of Splenic Lymphoma with Villous Lymphocytes

Abstract

We report here a case of “splenic lymphoma with villous lymphocytes” (SLVL) which exhibited both B- and T-cell phenotypes and genotypes. The patient was a 73-year-old man. Physical examination revealed splenomegaly and lymphadenopathy. The white blood cell count was 55.2 × 10⁹/L with 70.5% atypical lymphocytes, having cytoplasmic villi, characteristic of SLVL. The atypical cells infiltrated both the red and white pulps. Immunological analysis of the peripheral leukocytes showed both B- and T-cell phenotypes (CD5, CD19, CD20, HLA-DR, SmIgM and λ positive). DNA analysis revealed a dual rearrangement of the immunoglobulin heavy chain gene and T-cell receptor β gene. SLVL has been identified as a B-cell leukemia with a relatively benign clinical course. This case had both B- and T-cell pheno- and genotypes with a progressive course. To the best of our knowledge, no case of SLVL with dual genotypes has ever been reported.

Key Words:

• hairy cell leukemia
• splenic lymphoma with villous lymphocytes
• IgH gene
• TCRβ gene