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. Am J Clin Pathol 1998 Jul;110(1):95-9
Date
07/14/1998Pubmed ID
9661927DOI
10.1093/ajcp/110.1.95Scopus ID
2-s2.0-0031780282 (requires institutional sign-in at Scopus site) 19 CitationsAbstract
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.
Author List
Kroft SH, Finn WG, Singleton TP, Ross CW, Sheldon S, Schnitzer BAuthor
Steven Howard Kroft MD Chair, Professor in the Pathology department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
Blotting, SouthernGene Rearrangement
Genes, Immunoglobulin
Herpesvirus 4, Human
Humans
Immunocompromised Host
Immunophenotyping
In Situ Hybridization
Infant
Karyotyping
Lymph Nodes
Lymphoma, Follicular
Lymphoma, Large-Cell, Immunoblastic
Male
Proto-Oncogene Proteins c-myc
Wiskott-Aldrich Syndrome