EBV-associated lymphoproliferative syndrome with a distinct 69 base-pair deletion in the LMP-1 oncogene.
We describe an immunocompetent 12-year-old boy with chronic EBV infection and lymphoid interstitial pneumonitis. Lymph node biopsies showed effacement of the architecture with polymorphic cellular infiltrates, consisting predominantly of T cells and natural killer cells. No clonal rearrangement of TCR or immunoglobulin genes was seen. DNA was extracted from hilar lymph nodes; sequencing of the carboxy terminal region of the latent membrane protein 1 (LMP-1) oncogene revealed a 69 base-pair deletion and four point mutations. Immunosuppressive treatment with prednisone and cyclosporine reversed the lymphadenopathy.
University Children's Hospital, Freiburg, Germany.
C
C
Klein
S
S
Rothenberger
C
C
Niemeyer
E
E
Bachmann
B
B
Odermatt
N
N
Böhm
M
M
Brandis
H
H
Knecht
D001482:Base Composition; D001483:Base Sequence; D002648:Child; D016572:Cyclosporine / Q000627:therapeutic use; D017931:DNA Primers / Q000235:genetics; D004587:Electrophoresis, Agar Gel; D017353:Gene Deletion; D006566:Herpesviridae Infections / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology; D004854:Herpesvirus 4, Human / Q000276:immunology*; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D008198:Lymph Nodes / Q000473:pathology; D008297:Male; D008969:Molecular Sequence Data; D017354:Point Mutation; D016133:Polymerase Chain Reaction; D011239:Prednisolone / Q000627:therapeutic use; D014412:Tumor Virus Infections / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology; D014763:Viral Matrix Proteins / Q000235:genetics*
D017931:DNA Primers; C037662:EBV-associated membrane antigen, Epstein-Barr virus; D014763:Viral Matrix Proteins; D016572:Cyclosporine; D011239:Prednisolone