title: Cytotoxic large T-cell lymphoma with fulminant clinical course, CD8+ and CD56- phenotype, and its relation to Epstein-Barr virus: a report of two cases.
abstract: Currently, the immunohistochemical evaluation of cytotoxic granule-associated proteins such as TIA-1 and granzyme B can be carried out on paraffin sections. This procedure has broadened our knowledge of cytotoxic lymphoid neoplasms. Their detection is now regarded as a useful adjunctive in some characterizations of cytotoxic T- or natural killer (NK)-cell lymphoma, mostly in lymphoma of extranodal origin. We report two cases of nodal cytotoxic large T-cell lymphoma with identical biologic properties. Both cases presented with systemic lymphadenopathy, lymphomatous bone marrow involvement, and thrombocytopenia. The clinical course was fulminant, and both patients died within 1 week of presentation. The cells had a characteristic immunophenotype of CD2+, CD3+, CD4-, CD5-, CD8+, CD30 -/+, CD56-, CD57-, TCR alpha/beta+, and TCR gamma/delta-. They also expressed the cytotoxic granule-associated proteins of TIA-1 and granzyme B, and exhibited clonal rearrangements of the T-cell receptor beta chain gene. Monoclonal integration of Epstein-Barr virus was also detected. The present cases exhibited clinicopathological features that were distinct from other types of malignant lymphoma expressing cytotoxic granule-associated proteins.
authors.affiliation: Department of Hematology and Chemotherapy, Aichi Cancer Center Hospital, Nagoya, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Kagami
authors.forename: R
authors.initials: R
authors.lastname: Sobue
authors.forename: N
authors.initials: N
authors.lastname: Ito
authors.forename: Y
authors.initials: Y
authors.lastname: Yatabe
authors.forename: H
authors.initials: H
authors.lastname: Taji
authors.forename: R
authors.initials: R
authors.lastname: Suzuki
authors.forename: M
authors.initials: M
authors.lastname: Seto
authors.forename: M
authors.initials: M
authors.lastname: Ogura
authors.forename: T
authors.initials: T
authors.lastname: Suchi
authors.forename: Y
authors.initials: Y
authors.lastname: Morishima
authors.forename: S
authors.initials: S
authors.lastname: Nakamura
mesh_terms: D000328:Adult; D000368:Aged; D015703:Antigens, CD / Q000276:immunology; D015496:CD4-Positive T-Lymphocytes / Q000276:immunology* / Q000473:pathology / Q000821:virology*; D018414:CD8-Positive T-Lymphocytes / Q000276:immunology* / Q000473:pathology / Q000821:virology*; D003602:Cytotoxicity, Immunologic; D004854:Herpesvirus 4, Human / Q000276:immunology*; D006801:Humans; D016130:Immunophenotyping; D007694:Killer Cells, Natural / Q000276:immunology; D016399:Lymphoma, T-Cell / Q000276:immunology* / Q000473:pathology / Q000821:virology; D008297:Male
chemical_list: D015703:Antigens, CD