Journal
JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY
Volume 37, Issue 2, Pages E114-E117Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/MPH.0b013e31829f3496
Keywords
extranodal marginal zone lymphoma; ataxia telangiectasia; Epstein-Barr virus
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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.
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