title: Follicular large cell lymphoma with immunoblastic features in a child with Wiskott-Aldrich syndrome: an unusual immunodeficiency-related neoplasm not associated with Epstein-Barr virus.
abstract: Patients with Wiskott-Aldrich syndrome, a severe inherited immunodeficiency disorder, have a markedly increased risk of developing non-Hodgkin's lymphoma compared with the general population. These are uniformly diffuse aggressive B-cell neoplasms that resemble those seen in AIDS and the posttransplantation setting and also may be associated with Epstein-Barr virus. We report what to our knowledge is the first case of follicular lymphoma in a 14-year-old child with Wiskott-Aldrich syndrome. The neoplasm was composed predominantly of large cells with immunoblastic 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 hybridization and Southern blot terminal repeat analysis. Interestingly, however, the tumor contained c-myc gene rearrangement.
authors.affiliation: University of Michigan Medical School, Ann Arbor, USA.
authors.forename: S H
authors.initials: SH
authors.lastname: Kroft
authors.forename: W G
authors.initials: WG
authors.lastname: Finn
authors.forename: T P
authors.initials: TP
authors.lastname: Singleton
authors.forename: C W
authors.initials: CW
authors.lastname: Ross
authors.forename: S
authors.initials: S
authors.lastname: Sheldon
authors.forename: B
authors.initials: B
authors.lastname: Schnitzer
mesh_terms: D015139:Blotting, Southern; D015321:Gene Rearrangement; D005803:Genes, Immunoglobulin; D004854:Herpesvirus 4, Human / Q000302:isolation & purification; D006801:Humans; D016867:Immunocompromised Host; D016130:Immunophenotyping; D017403:In Situ Hybridization; D007223:Infant; D007621:Karyotyping; D008198:Lymph Nodes / Q000473:pathology; D008224:Lymphoma, Follicular / Q000150:complications* / Q000235:genetics / Q000473:pathology; D016400:Lymphoma, Large-Cell, Immunoblastic / Q000150:complications* / Q000235:genetics / Q000473:pathology; D008297:Male; D016271:Proto-Oncogene Proteins c-myc / Q000235:genetics; D014923:Wiskott-Aldrich Syndrome / Q000150:complications*
chemical_list: D016271:Proto-Oncogene Proteins c-myc