BCL-6 protein is expressed in precursor T-cell lymphoblastic lymphoma and in prenatal and postnatal thymus

Blood. 2001 Jan 1;97(1):270-6. doi: 10.1182/blood.v97.1.270.

Abstract

The organization and expression of the BCL-6 gene in normal and neoplastic thymic T cells has not been fully determined. We examined 8 precursor T-cell lymphoblastic lymphomas (T-LBLs) and found significant BCL-6 expression in 4 cases. Three of the BCL-6(+) cases expressed a common thymocyte phenotype (CD4(+), CD8(+)), and one expressed a precursor thymocyte phenotype (CD4(-), CD8(-)). In 6 cases evaluated, including those expressing BCL-6, molecular analyses demonstrated a germline configuration of the BCL-6 gene and a wild-type BCL-6 gene first exon-intron boundary region. We also evaluated 12 normal prenatal and postnatal thymuses for BCL-6 protein. BCL-6 was expressed by most cortical thymocytes and by scattered medullary thymocytes. BCL-6(+) cortical and medullary thymocytes also expressed CD2, CD3, CD4, CD5, CD7, or CD8. We further analyzed the pattern of BCL-2 and BCL-X(L) expression and their coexpression with BCL-6 in normal thymus and T-LBL and compared it to that of follicle centers of reactive lymph nodes and follicular lymphoma. BCL-6(+) cortical thymocytes coexpressed BCL-X(L) but not BCL-2. All 4 BCL-6(+) T-LBLs and 4 BCL-6(-) T-LBLs coexpressed BCL-2 and BCL-X(L). Conceivably, T-LBLs may arise through clonal expansion of cortical thymocytes normally expressing the BCL-6 protein. The pattern of BCL-6, BCL-2, and BCL-X(L) expression in cortical thymocytes is highly reminiscent of germinal centers, and the abnormal coexpression of BCL-2, BCL-X(L), and BCL-6 in T-LBL is analogous to coexpression in follicle center cell lymphomas, suggesting that coexpression of these anti-apoptotic genes may contribute to the pathogenesis of T-LBL.

Publication types

  • Comparative Study

MeSH terms

  • Adolescent
  • Adult
  • Apoptosis / drug effects
  • Child
  • Child, Preschool
  • DNA-Binding Proteins / metabolism*
  • Female
  • Fetus / chemistry
  • Fetus / cytology
  • Humans
  • Immunophenotyping
  • Infant
  • Infant, Newborn
  • Lymphoma, Follicular / etiology
  • Lymphoma, Follicular / metabolism
  • Lymphoma, T-Cell / etiology
  • Lymphoma, T-Cell / metabolism
  • Lymphoma, T-Cell / pathology
  • Male
  • Middle Aged
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / etiology
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / metabolism
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / pathology
  • Proto-Oncogene Proteins / metabolism*
  • Proto-Oncogene Proteins c-bcl-2 / metabolism
  • Proto-Oncogene Proteins c-bcl-6
  • Thymus Gland / chemistry
  • Thymus Gland / embryology*
  • Thymus Gland / pathology
  • Transcription Factors / metabolism*
  • bcl-X Protein

Substances

  • BCL2L1 protein, human
  • DNA-Binding Proteins
  • Proto-Oncogene Proteins
  • Proto-Oncogene Proteins c-bcl-2
  • Proto-Oncogene Proteins c-bcl-6
  • Transcription Factors
  • bcl-X Protein