FOLLICULAR LARGE-CELL LYMPHOMA WITH IMMUNOBLASTIC FEATURES IN A CHILDWITH WISKOTT-ALDRICH-SYNDROME - AN UNUSUAL IMMUNODEFICIENCY-RELATED NEOPLASM NOT ASSOCIATED WITH EPSTEIN-BARR-VIRUS

Patients with Wiskott-Aldrich syndrome, a severe inherited immunodefic iency disorder, have a markedly increased risk of developing non-Hodgk in's lymphoma compared with the general population. These are uniforml y diffuse aggressive B-cell neoplasms that resemble those seen in AIDS and the posttransplantation setting and also may be associated with E pstein-Barr virus. We report tt!hat to our knowledge is the first case of follicular lymphoma in a 14-year-old child with Wiskott-Aldrich sy ndrome. The neoplasm was composed predominantly of large cells with im munoblastic features, and it possessed light chain-restricted surface immunoglobulin, clonal immunoglobulin gene rearrangements, and a t(14; 18). The tumor lacked Epstein-Barr virus sequences by in situ hybridiz ation and Southern blot terminal repent analysis. Interestingly, howev er the tumor contained c-myc gene rearrangement.