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.
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.
Tanaka
Y
Y
Department of Pathology, Kanagawa Children's Medical Center, Yokohama, Japan.
Sasaki
Y
Y
Kurozumi
H
H
Hyodo
Y
Y
Nishi
T
T
Nakatani
Y
Y
Imai
S
S
Osato
T
T
D001808:Blood Vessels / Q000473:pathology*; D002051:Burkitt Lymphoma / Q000150:complications* / Q000473:pathology*; D015704:CD4 Antigens / Q000032:analysis; D002455:Cell Division; D002648:Child; D002908:Chronic Disease; D002999:Clone Cells; D004854:Herpesvirus 4, Human / Q000302:isolation & purification; D006801:Humans; D007160:Immunoproliferative Disorders / Q000150:complications* / Q000473:pathology*; D008297:Male; D013601:T-Lymphocytes / Q000276:immunology / Q000382:microbiology
D015704:CD4 Antigens