Peripheral T-cell lymphoma with involvement of the expanded mantle zone

Virchows Arch. 2006 Jul;449(1):78-87. doi: 10.1007/s00428-005-0123-z. Epub 2006 Apr 22.

Abstract

Peripheral T-cell lymphoma (PTCL) with a nodular architecture is rare. Recently, two variants have been described with infiltration of the B-cell follicle, one variant that localizes to the marginal zone with a so-called perifollicular growth pattern, and a variant that localizes to the germinal center. These lymphomas have a CD4+ phenotype and may express Bcl-6. We have studied five similar cases of PTCL with involvement of the B-cell follicle. However, our cases differ from the cases previously described by their predominant and frequently patchy involvement of the expanded mantle zone of the B-cell follicle at onset. Later biopsies in three of the cases show diffuse infiltration of the lymph node, without features of angioimmunoblastic TCL (AILT). All cases expressed Bcl-6 in addition to CD4. Cytogenetics was available in four of the cases but revealed no recurrent chromosomal aberrations or changes associated with other types of PTCL. No mutations of the BCL-6 gene were observed. Together, the cases seem to have an intermediately aggressive clinical behavior. Whether our cases are part of a spectrum of PTCLs that encompasses previously described variants with predominant marginal zone or germinal center infiltration or they represent a separate T-cell lymphoma type remains to be demonstrated by a study of more of such cases.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Biomarkers, Tumor / metabolism
  • CD4 Antigens / metabolism
  • Combined Modality Therapy
  • DNA-Binding Proteins / metabolism
  • Fatal Outcome
  • Female
  • Germinal Center / pathology
  • Humans
  • Lymph Nodes / pathology*
  • Lymphoma, Mantle-Cell / metabolism
  • Lymphoma, Mantle-Cell / pathology*
  • Lymphoma, Mantle-Cell / therapy
  • Lymphoma, T-Cell, Peripheral / metabolism
  • Lymphoma, T-Cell, Peripheral / pathology*
  • Lymphoma, T-Cell, Peripheral / therapy
  • Male
  • Middle Aged
  • Proto-Oncogene Proteins c-bcl-6
  • Remission Induction

Substances

  • BCL6 protein, human
  • Biomarkers, Tumor
  • CD4 Antigens
  • DNA-Binding Proteins
  • Proto-Oncogene Proteins c-bcl-6