T-cell receptor delta/alpha rearrangements in lymphoid neoplasms

Blood. 1989 Aug 15;74(3):1073-83.

Abstract

Rearrangements within the T-cell receptor (TCR)delta/alpha locus were analyzed in a wide variety of lymphoid neoplasms by eight DNA probes specific for TCR J delta, J alpha and C alpha segments. In all 11 T-cell malignancies, rearrangement and/or deletion of TCR delta was detected irrespective of the stage of maturation of the tumor. The organization of TCR delta correlated with the phenotype of the tumor: In "prethymic" T-cell acute lymphocytic leukemia (ALL), TCR delta was the only TCR gene to be rearranged. More mature T cell malignancies expressing CD4 together with CD3 showed deletion of both alleles of TCR delta, suggestive of TCR V alpha-J alpha rearrangement. All 43 B-cell tumors expressing surface immunoglobulin (sIg), including two cases of adult B-cell ALL, had germline configuration of TCR delta/alpha. In contrast, all 17 B-cell precursor ALLs (null, common, and pre-B-cell ALLs) had rearrangement and/or deletion of TCR delta/alpha. A single case of "histiocytic" lymphoma also showed biallelic deletion of TCR delta. Oligoclonal rearrangements of Ig and TCR genes were observed in two cases of B-cell precursor ALL and in one case of T-cell lymphoblastic lymphoma. Patterns of such "aberrant" TCR rearrangement were similar to those observed in T-lineage malignancies. In particular, seven of eight cases of B-cell precursor ALL and the histiocytic lymphoma which demonstrated biallelic TCR delta deletion, (suggestive of a V alpha-J alpha rearrangement) had clonal TCR beta rearrangement. These data support the hypothesis that supposedly aberrant rearrangements of the TCR genes may follow the same developmental controls as found in T-cell differentiation, despite the lack of evidence for further commitment to the T-cell lineage. TCR delta rearrangement is a useful marker of clonality of immature T-cell tumors which may have only this gene rearranged but is not specific to the T-cell lineage.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Antigens, Differentiation, T-Lymphocyte / analysis
  • Burkitt Lymphoma / genetics
  • Burkitt Lymphoma / immunology
  • Burkitt Lymphoma / metabolism
  • Child
  • Cytoplasm / analysis
  • Gene Rearrangement, alpha-Chain T-Cell Antigen Receptor*
  • Genes, Immunoglobulin
  • Humans
  • Leukemia / classification
  • Leukemia / genetics*
  • Leukemia, B-Cell / classification
  • Leukemia, B-Cell / genetics
  • Leukemia, T-Cell / classification
  • Leukemia, T-Cell / genetics
  • Lymphoma / classification
  • Lymphoma / genetics*
  • Lymphoma, Large B-Cell, Diffuse / genetics
  • Neoplastic Stem Cells / analysis
  • Phenotype
  • Receptors, Antigen, B-Cell / analysis
  • Receptors, Antigen, T-Cell / genetics*

Substances

  • Antigens, Differentiation, T-Lymphocyte
  • Receptors, Antigen, B-Cell
  • Receptors, Antigen, T-Cell