title: Aggressive Epstein-Barr virus-associated, CD8+, CD30+, CD56+, surface CD3-, natural killer (NK)-like cytotoxic T-cell lymphoma.
abstract: We report an unusual case of aggressive natural killer (NK)-like cytotoxic T-cell lymphoma in a previously healthy immunocompetent West African male. He presented with a fever of unknown origin, subsequently developed erythematous skin nodules, generalized lymphadenopathy, and hepatosplenomegaly, and then died of multiple organ failure. A skin nodule and lymph node biopsy showed an infiltrate of pleomorphic atypical medium and large lymphoid cells with extensive necrosis and prominent apoptosis. Peripheral blood and ascites also harbored these cells, with cytology revealing irregular nuclear folding and basophilic cytoplasm, and some with azurophilic cytoplasmic granules. Flow cytometry and immunohistochemistry demonstrated the expression of CD2, CD7, CD8, CD30, CD56, and cytoplasmic but not surface CD3. In situ hybridization demonstrated Epstein-Barr virus transcripts. A monoclonal T-cell receptor gamma chain gene rearrangement was detected by polymerase chain reaction. This is the first reported case of an NK-like T-cell lymphoma with these unusual features, making precise classification difficult. Some features suggest an NK1.1 or NKT lymphocyte origin. Because the earliest clinical manifestation was splenomegaly and abnormal liver function, the normal cellular counterpart may be a distinct subset of NK1.1 cells normally present in hepatosplenic sinusoids. This tumor disseminated early and pursued a fulminant clinical course, thus emphasizing the importance of early recognition and diagnosis.
authors.affiliation: Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA.
authors.forename: Jianguo
authors.initials: J
authors.lastname: Tao
authors.forename: Suresh G
authors.initials: SG
authors.lastname: Shelat
authors.forename: Elaine S
authors.initials: ES
authors.lastname: Jaffe
authors.forename: Adam
authors.initials: A
authors.lastname: Bagg
mesh_terms: D000328:Adult; D017252:CD3 Complex; D019002:CD56 Antigen; D016827:CD8 Antigens; D020031:Epstein-Barr Virus Infections / Q000276:immunology / Q000473:pathology; D017809:Fatal Outcome; D006801:Humans; D017730:Ki-1 Antigen; D007694:Killer Cells, Natural / Q000276:immunology / Q000473:pathology*; D008198:Lymph Nodes / Q000473:pathology; D016410:Lymphoma, T-Cell, Cutaneous / Q000276:immunology / Q000473:pathology; D008297:Male; D009102:Multiple Organ Failure / Q000209:etiology; D012878:Skin Neoplasms / Q000276:immunology / Q000473:pathology
chemical_list: D017252:CD3 Complex; D019002:CD56 Antigen; D016827:CD8 Antigens; D017730:Ki-1 Antigen