Hepatosplenic gammadelta T-cell lymphoma is a rare clinicopathologic entity with poor outcome: report on a series of 21 patients.
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.
Belhadj
Karim
K
Département de Pathologie and EA2348, CHU Henri Mondor, 94010, Créteil, France.
Reyes
Felix
F
Farcet
Jean-Pierre
JP
Tilly
Herve
H
Bastard
Christian
C
Angonin
Regis
R
Deconinck
Eric
E
Charlotte
Frederic
F
Leblond
Veronique
V
Labouyrie
Eric
E
Lederlin
Pierre
P
Emile
Jean-Francois
JF
Delmas-Marsalet
Beatrice
B
Arnulf
Bertrand
B
Zafrani
Elie-Serge
ES
Gaulard
Philippe
P
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
D016692:Receptors, Antigen, T-Cell, gamma-delta