title: Nonhepatosplenic γδ T-cell lymphomas represent a spectrum of aggressive cytotoxic T-cell lymphomas with a mainly extranodal presentation.
abstract: γδ T cells represent a minor T-cell subset that is mainly distributed in mucosal surfaces. Two distinct lymphomas derived from these cells have been recognized: hepatosplenic γδ T-cell lymphoma (HSTL) and primary cutaneous γδ T-cell lymphoma (PCGD-TCL). However, whether other anatomic sites may also be involved and whether they represent a spectrum of the same disease are not well studied. The lack of T-cell receptor (TCR)β expression has been used to infer a γδ origin when other methods are not available. We studied 35 T-cell tumors suspected to be γδ TCL using monoclonal antibodies reactive with TCR δ or γ in paraffin sections. We were able to confirm γδ chain expression in 22 of 35 cases. We identified 8 PCGD-TCLs, 6 HSTLs, and 8 γδ TCLs without hepatosplenic or cutaneous involvement involving mainly extranodal sites. Two such cases were classified as enteropathy-associated T-cell lymphoma, type II. The other γδ TCL presented in the intestine, lung, tongue, orbit, and lymph node. In addition, we observed 13 cases with mainly extranodal involvement that lacked any TCR expression ("TCR silent"). In all cases, a natural killer cell origin was excluded. In conclusion, the lack of TCRβ expression does not always predict γδ-T-cell derivation, as TCR silent cases may be found. The recognition of γδ TCL presenting in extranodal sites other than skin and liver/spleen expands the clinical spectrum of these tumors. However, non-HSTL γδ TCL do not seem to represent a single entity. The relationship of these tumors with either HSTL or PCGD-TCL requires further study.
authors.affiliation: Department of Pathology, Hospital Clinic, IDIBAPS, Barcelona, Spain.
authors.forename: Adriana
authors.initials: A
authors.lastname: Garcia-Herrera
authors.forename: Joo Y
authors.initials: JY
authors.lastname: Song
authors.forename: Shih-Sung
authors.initials: SS
authors.lastname: Chuang
authors.forename: Neus
authors.initials: N
authors.lastname: Villamor
authors.forename: Luis
authors.initials: L
authors.lastname: Colomo
authors.forename: Stefania
authors.initials: S
authors.lastname: Pittaluga
authors.forename: Tomas
authors.initials: T
authors.lastname: Alvaro
authors.forename: Maria
authors.initials: M
authors.lastname: Rozman
authors.forename: Jazmin
authors.initials: J
authors.lastname: de Anda Gonzalez
authors.forename: Ana Maria
authors.initials: AM
authors.lastname: Arrunategui
authors.forename: Eva
authors.initials: E
authors.lastname: Fernandez
authors.forename: Elena
authors.initials: E
authors.lastname: Gonzalvo
authors.forename: Teresa
authors.initials: T
authors.lastname: Estrach
authors.forename: Dolors
authors.initials: D
authors.lastname: Colomer
authors.forename: Mark
authors.initials: M
authors.lastname: Raffeld
authors.forename: Philippe
authors.initials: P
authors.lastname: Gaulard
authors.forename: Elias
authors.initials: E
authors.lastname: Campo
authors.forename: Elaine S
authors.initials: ES
authors.lastname: Jaffe
authors.forename: Antonio
authors.initials: A
authors.lastname: Martinez
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor; D019675:Genes, T-Cell Receptor gamma; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D017403:In Situ Hybridization; D016399:Lymphoma, T-Cell / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D016410:Lymphoma, T-Cell, Cutaneous / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D016612:Paraffin Embedding; D016133:Polymerase Chain Reaction; D016693:Receptors, Antigen, T-Cell, alpha-beta / Q000032:analysis; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000032:analysis* / Q000235:genetics; D012878:Skin Neoplasms / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D013602:T-Lymphocytes, Cytotoxic / Q000276:immunology* / Q000473:pathology; D055815:Young Adult
chemical_list: D016693:Receptors, Antigen, T-Cell, alpha-beta; D016692:Receptors, Antigen, T-Cell, gamma-delta