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 infect ion and lymphoid interstitial pneumonitis. Lymphnode biopsies showed e ffacement of the architecture with polymorphic cellular infiltrates, c onsisting predominantly of T cells and natural killer cells. No clonal rearrangement of TCR or immunoglobulin genes was seen, DNA was extrac ted from hilar lymph nodes; sequencing of the carboxy terminal region of the latent membrane protein 1 (LMP-1) oncogene revealed a 69 base-p air deletion and four point mutations. Immunosuppressive treatment wit h prednisone and cyclosporine reversed the lymphadenopathy.