Angiocentric immunoproliferative lesion associated with chronic active Epstein-Barr virus infection in an 11-year-old boy. Clonotopic proliferation of Epstein-Barr virus-bearing CD4+ T lymphocytes

Am J Surg Pathol. 1994 Jun;18(6):623-31. doi: 10.1097/00000478-199406000-00006.


We report a pulmonary angiocentric immunoproliferative lesion (AIL) in an 11-year-old boy with chronic active Epstein-Barr virus (EBV) infection. The phenotypes of the proliferating lymphoid cells in the biopsied pulmonary lesion were CD2+, CD3+, CD4+, CD5+, CD7+, and HLA-DR+. EBV DNA was detected in the tumorous and the nontumorous tissue by Southern-blotting studies. Dual immunostains and combined immunohistochemistry/in situ hybridization showed the simultaneous presence of EBV-determined nuclear antigen or EBV-encoded small RNAs and T-cell markers in the lymphoid cells. Molecular genetic analysis of the tumorous lesion diagnosed as AIL grade III showed no clonal rearrangement of the T-cell receptor beta gene but a single type of fused terminal band of EBV. No such evidence of monoclonality was identified in the surrounding nontumorous tissue diagnosed as AIL grade I or II. The present case was a rare example of AIL in childhood and provides further histopathologic and molecular biological evidence supporting the concept of AIL as a continuous spectrum from premalignant lymphoproliferative disorders to monoclonal, overt malignant lymphoma.

Publication types

  • Case Reports

MeSH terms

  • Blood Vessels / pathology*
  • Burkitt Lymphoma / complications*
  • Burkitt Lymphoma / pathology*
  • CD4 Antigens / analysis
  • Cell Division
  • Child
  • Chronic Disease
  • Clone Cells
  • Herpesvirus 4, Human* / isolation & purification
  • Humans
  • Immunoproliferative Disorders / complications*
  • Immunoproliferative Disorders / pathology*
  • Male
  • T-Lymphocytes / immunology
  • T-Lymphocytes / microbiology


  • CD4 Antigens