title: Hepatosplenic gammadelta T-cell lymphoma is a rare clinicopathologic entity with poor outcome: report on a series of 21 patients.
abstract: We report on the characteristics of 21 patients with hepatosplenic gammadelta T-cell lymphoma (HSgammadeltaTCL), an entity recognized since 1994 in the Revised European American Lymphoma (REAL) classification. Median age was 34 years. Patients had splenomegaly (n = 21), hepatomegaly (n = 15), and thrombocytopenia (n = 20). Histopathologic findings were homogeneous and showed the presence of medium-sized lymphoma cells within the sinusoids of splenic red pulp, liver, and bone marrow. Marrow involvement was usually mild but could be demonstrated by phenotyping in all patients. Cells were CD3+CD5-, expressed the gammadelta T-cell receptor, and had a nonactivated cytotoxic cell phenotype (TIA-1+, granzyme B-). Most patients were CD4-/CD8- (16 of 18); CD56+ (15 of 18), expressed the Vdelta1epitope (Vd1+/Vd2-/Vd3-) (9 of 12); and were negative for Epstein-Barr virus (EBV) (18 of 20). Isochromosome arm 7q was documented in 9 of 13 patients. Eight patients had previously undergone kidney transplantation or had a history of systemic lupus, Hodgkin disease, or malaria. Prognosis was poor; median survival time was 16 months, and all but 2 patients ultimately died despite consolidative or salvage high-dose therapy. In conclusion, HSgammadeltaTCL is a disease with distinctive clinical, histopathologic, and phenotypic characteristics. Bone marrow biopsy with combined phenotyping is sufficient for diagnosis, and splenectomy is therefore unwarranted. Current treatment modalities appear to be ineffective in most patients.
authors.affiliation: Département de Pathologie and EA2348, CHU Henri Mondor, 94010, Créteil, France.
authors.forename: Karim
authors.initials: K
authors.lastname: Belhadj
authors.forename: Felix
authors.initials: F
authors.lastname: Reyes
authors.forename: Jean-Pierre
authors.initials: JP
authors.lastname: Farcet
authors.forename: Herve
authors.initials: H
authors.lastname: Tilly
authors.forename: Christian
authors.initials: C
authors.lastname: Bastard
authors.forename: Regis
authors.initials: R
authors.lastname: Angonin
authors.forename: Eric
authors.initials: E
authors.lastname: Deconinck
authors.forename: Frederic
authors.initials: F
authors.lastname: Charlotte
authors.forename: Veronique
authors.initials: V
authors.lastname: Leblond
authors.forename: Eric
authors.initials: E
authors.lastname: Labouyrie
authors.forename: Pierre
authors.initials: P
authors.lastname: Lederlin
authors.forename: Jean-Francois
authors.initials: JF
authors.lastname: Emile
authors.forename: Beatrice
authors.initials: B
authors.lastname: Delmas-Marsalet
authors.forename: Bertrand
authors.initials: B
authors.lastname: Arnulf
authors.forename: Elie-Serge
authors.initials: ES
authors.lastname: Zafrani
authors.forename: Philippe
authors.initials: P
authors.lastname: Gaulard
mesh_terms: D000293:Adolescent; D000328:Adult; D001853:Bone Marrow / Q000473:pathology; D002465:Cell Movement; D002869:Chromosome Aberrations; D002897:Chromosomes, Human, Pair 7 / Q000648:ultrastructure; D005260:Female; D006529:Hepatomegaly / Q000209:etiology* / Q000473:pathology; D004854:Herpesvirus 4, Human / Q000302:isolation & purification; D006801:Humans; D016867:Immunocompromised Host; D016130:Immunophenotyping; D016030:Kidney Transplantation; D016399:Lymphoma, T-Cell / Q000145:classification* / Q000401:mortality / Q000473:pathology / Q000628:therapy; D008288:Malaria / Q000150:complications; D008297:Male; D008875:Middle Aged; D014411:Neoplastic Stem Cells / Q000737:chemistry / Q000473:pathology*; D011183:Postoperative Complications / Q000473:pathology; D011379:Prognosis; D016692:Receptors, Antigen, T-Cell, gamma-delta / Q000032:analysis*; D012189:Retrospective Studies; D013163:Splenomegaly / Q000209:etiology* / Q000473:pathology; D016176:T-Lymphocyte Subsets / Q000737:chemistry / Q000473:pathology*; D013921:Thrombocytopenia / Q000209:etiology* / Q000473:pathology; D017211:Treatment Failure
chemical_list: D016692:Receptors, Antigen, T-Cell, gamma-delta