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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

07/14/1998

Pubmed ID

9661927

DOI

10.1093/ajcp/110.1.95

Scopus ID

2-s2.0-0031780282   17 Citations

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.

Author List

Kroft SH, Finn WG, Singleton TP, Ross CW, Sheldon S, Schnitzer B

Author

Steven Howard Kroft MD Chair, Professor in the Pathology department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Blotting, Southern
Gene 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