Nodal Epstein-Barr virus-positive T-cell/NK-cell lymphoma associated with immunodeficiency: a rare condition looking for recognition
Identifieur interne : 002775 ( Main/Exploration ); précédent : 002774; suivant : 002776Nodal Epstein-Barr virus-positive T-cell/NK-cell lymphoma associated with immunodeficiency: a rare condition looking for recognition
Auteurs : Cristiane Rúbia Ferreira ; Aloísio Felipe-Silva ; Fernando Peixoto Ferraz De Campos ; Maria Cláudia Nogueira ZerbiniSource :
- Autopsy & Case Reports [ 2236-1960 ] ; 2014.
Abstract
The authors describe a peculiar form of Epstein-Barr virus (EBV)-associated T-cell lymphoma in an HIV-positive patient presenting an aggressive clinical course. Unlike most other EBV-positive T-cell/natural-killer (NK)-cell lymphomas, the disease was characterized by predominant nodal involvement at presentation. T-cell lineage was confirmed by T-cell receptor-rearrangement, and neoplastic cells exhibited strong and diffuse CD56 expression. A marked intravascular component was detected in the skin, the liver, and the lung parenchyma. This entity was not predicted in the WHO 2008 classification, but has been recently identified in immunocompromised patients. This case report refers to a middle-aged man with AIDS, who presented a 4-month history of weight loss, fever, hepatosplenomegaly, peripheral and deep-chain lymphadenopathy. A blood smear showed lymphocytosis with a marked presence of atypia. The outcome was unfavorable and the patient could not be treated. The autopsy revealed multivisceral involvement, including lymph nodes, spleen, bone marrow, liver, lungs, skin, and kidneys.
Url:
DOI: 10.4322/acr.2014.036
PubMed: 28573126
PubMed Central: 5443130
Affiliations:
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first="Aloísio" last="Felipe-Silva">Aloísio Felipe-Silva</name><affiliation><nlm:aff id="aff01">Anatomic Pathology Service – Hospital Universitário –<institution content-type="orgname">Universidade de São Paulo</institution>, São Paulo/SP – Brazil.</nlm:aff><wicri:noCountry code="subfield">São Paulo/SP – Brazil.</wicri:noCountry></affiliation></author><author><name sortKey="De Campos, Fernando Peixoto Ferraz" sort="De Campos, Fernando Peixoto Ferraz" uniqKey="De Campos F" first="Fernando Peixoto Ferraz" last="De Campos">Fernando Peixoto Ferraz De Campos</name><affiliation><nlm:aff id="aff02">Department of Internal Medicine – Hospital Universitário –<institution content-type="orgname">Universidade de São Paulo</institution>, São Paulo/SP – Brazil.</nlm:aff><wicri:noCountry code="subfield">São Paulo/SP – Brazil.</wicri:noCountry></affiliation></author><author><name sortKey="Zerbini, Maria Claudia Nogueira" sort="Zerbini, Maria Claudia Nogueira" uniqKey="Zerbini M" first="Maria Cláudia Nogueira" last="Zerbini">Maria Cláudia Nogueira Zerbini</name><affiliation><nlm:aff id="aff03">Department of Pathology – Faculdade de Medicina –<institution content-type="orgname">Universidade de São Paulo</institution>, São Paulo/SP – Brazil.</nlm:aff><wicri:noCountry code="subfield">São Paulo/SP – Brazil.</wicri:noCountry></affiliation></author></analytic><series><title level="j">Autopsy & Case Reports</title><idno type="eISSN">2236-1960</idno><imprint><date when="2014">2014</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><p>The authors describe a peculiar form of Epstein-Barr virus (EBV)-associated T-cell lymphoma in an HIV-positive patient presenting an aggressive clinical course. Unlike most other EBV-positive T-cell/natural-killer (NK)-cell lymphomas, the disease was characterized by predominant nodal involvement at presentation. T-cell lineage was confirmed by T-cell receptor-rearrangement, and neoplastic cells exhibited strong and diffuse CD56 expression. A marked intravascular component was detected in the skin, the liver, and the lung parenchyma. This entity was not predicted in the WHO 2008 classification, but has been recently identified in immunocompromised patients. This case report refers to a middle-aged man with AIDS, who presented a 4-month history of weight loss, fever, hepatosplenomegaly, peripheral and deep-chain lymphadenopathy. A blood smear showed lymphocytosis with a marked presence of atypia. The outcome was unfavorable and the patient could not be treated. 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Asso-Bonnet</name></author><author><name sortKey="Raphael, M" uniqKey="Raphael M">M Raphael</name></author><author><name sortKey="Brousse, N" uniqKey="Brousse N">N Brousse</name></author></analytic></biblStruct></listBibl></div1></back></TEI><affiliations><list></list><tree><noCountry><name sortKey="De Campos, Fernando Peixoto Ferraz" sort="De Campos, Fernando Peixoto Ferraz" uniqKey="De Campos F" first="Fernando Peixoto Ferraz" last="De Campos">Fernando Peixoto Ferraz De Campos</name><name sortKey="Felipe Silva, Aloisio" sort="Felipe Silva, Aloisio" uniqKey="Felipe Silva A" first="Aloísio" last="Felipe-Silva">Aloísio Felipe-Silva</name><name sortKey="Ferreira, Cristiane Rubia" sort="Ferreira, Cristiane Rubia" uniqKey="Ferreira C" first="Cristiane Rúbia" last="Ferreira">Cristiane Rúbia Ferreira</name><name sortKey="Zerbini, Maria Claudia Nogueira" sort="Zerbini, Maria Claudia Nogueira" uniqKey="Zerbini M" first="Maria Cláudia Nogueira" last="Zerbini">Maria Cláudia Nogueira 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