title: Epstein-barr virus-associated extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT Lymphoma) arising in the parotid gland of a child with ataxia telangiectasia.
abstract: Hematologic malignancies, in particular T-cell lymphomas/leukemias, are prevalent in patients with ataxia telangiectasia (AT), with most reported cases being clinically aggressive and high grade. Epstein-Barr virus (EBV) is often associated with lymphoid proliferations/neoplasms arising in immunodeficient patients. Reports of low-grade B-cell neoplasms in the ataxia telangiectasia population are extremely rare. Here, we describe a case of EBV-associated extranodal marginal zone lymphoma (mucosa-associated lymphoid tissue lymphoma) of the parotid gland in a 16-year-old boy with AT. In addition, we review the literature of hematologic malignancies in the AT population as well as the occurrence of EBV in mucosa-associated lymphoid tissue lymphoma.
authors.affiliation: Department of Pathology, Penn State Milton S. Hershey Medical Center, Hershey, PA.
authors.forename: Jennifer A
authors.initials: JA
authors.lastname: Bennett
authors.forename: Michael G
authors.initials: MG
authors.lastname: Bayerl
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001260:Ataxia Telangiectasia / Q000150:complications* / Q000473:pathology / Q000821:virology; D020031:Epstein-Barr Virus Infections / Q000150:complications* / Q000821:virology; D004854:Herpesvirus 4, Human / Q000472:pathogenicity*; D006801:Humans; D018442:Lymphoma, B-Cell, Marginal Zone / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D008297:Male; D010306:Parotid Gland / Q000473:pathology* / Q000821:virology; D011379:Prognosis