Chronic lymphocytic leukemia with t(14;19)(q32;q13) is characterized by atypical morphologic and immunophenotypic features and distinctive genetic features

Am J Clin Pathol. 2011 May;135(5):686-96. doi: 10.1309/AJCPOEFP3SLX6HXJ.

Abstract

The t(14;19)(q32;q13) involving the IGH@ and BCL3 loci is an infrequent cytogenetic abnormality detected in B-cell malignancies. We describe the clinicopathologic, cytogenetic, and molecular genetic characteristics of 14 cases of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) with t(14;19)(q32;q13). All patients (10 men and 4 women) had lymphocytosis; 10 had lymphadenopathy. Blood and bone marrow lymphocytes were predominantly small, but cytologically and immunophenotypically atypical. In all cases, t(14;19) was found in the neoplastic stem line; it was the sole abnormality in 4. Ten cases showed additional cytogenetic abnormalities, including trisomy 12 in 9 and complex karyotypes in 7. Fluorescence in situ hybridization demonstrated IGH@/BCL3 fusion gene in all cases. In all cases, the IGHV genes were unmutated, but only 7 expressed ZAP70. Seven cases preferentially used IGHV4-39. Our results indicate that t(14;19)(q32;q13) identifies a subset of CLL/SLL with distinctive clinicopathologic and genetic features. Furthermore, t(14;19) may represent an early, possibly primary, genetic event.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • B-Lymphocytes / immunology
  • B-Lymphocytes / pathology*
  • Bone Marrow / pathology
  • Cell Size
  • Chromosomes, Human, Pair 14
  • Chromosomes, Human, Pair 19
  • Female
  • Humans
  • Immunophenotyping
  • In Situ Hybridization, Fluorescence
  • Leukemia, Lymphocytic, Chronic, B-Cell* / genetics
  • Leukemia, Lymphocytic, Chronic, B-Cell* / pathology
  • Leukemia, Lymphocytic, Chronic, B-Cell* / physiopathology
  • Lymphocytosis / genetics
  • Lymphocytosis / pathology
  • Male
  • Middle Aged
  • Mutation
  • Neoplastic Stem Cells / pathology
  • Oncogene Proteins, Fusion / genetics
  • Translocation, Genetic*
  • ZAP-70 Protein-Tyrosine Kinase / genetics

Substances

  • Oncogene Proteins, Fusion
  • ZAP-70 Protein-Tyrosine Kinase
  • ZAP70 protein, human