RESUMO
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.
Assuntos
Ataxia Telangiectasia/complicações , Infecções por Vírus Epstein-Barr/complicações , Herpesvirus Humano 4/patogenicidade , Linfoma de Zona Marginal Tipo Células B/diagnóstico , Glândula Parótida/patologia , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Ataxia Telangiectasia/patologia , Ataxia Telangiectasia/virologia , Infecções por Vírus Epstein-Barr/virologia , Humanos , Linfoma de Zona Marginal Tipo Células B/tratamento farmacológico , Linfoma de Zona Marginal Tipo Células B/etiologia , Masculino , Glândula Parótida/virologia , PrognósticoRESUMO
BACKGROUND: Human herpesvirus 6 (HHV6) is the etiologic agent of exanthem subitum. The virus is latent in salivary glands and saliva is the main form of viral transmission. The objective of this study was to assess HHV6 incidence in the fluids from healthy individuals using a standardised technique for collecting and extracting viral DNA from gingival crevicular fluid, whole saliva and parotid gland saliva. DESIGN: Samples of oral fluids and peripheral blood were collected from 28 blood donors and HHV6 was detected using PCR assay. Parotid gland saliva and gingival crevicular fluid were collected by endodontic paper cones in order to not contaminate these fluids with whole saliva. RESULTS: Of the 28 donors, 20 (71.4%) presented positive results in at least one of the three oral fluids researched. Whole saliva was positive in 19 (67.8%) volunteers, while only four (14.2%) samples of gingival crevicular fluid and four of parotid gland saliva proved to be positive. CONCLUSIONS: The results suggest that HHV6 is present in the saliva of a large proportion of the healthy adult population. The use of endodontic paper cones for oral fluid collection and viral extraction was efficient, simple, cheap and painless. In spite of, the small number of cases studied it was possible to demonstrate that neither gingival crevicular fluid nor parotid gland saliva were the principal source of HHV6 in whole saliva.