Hepatosplenic 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 T-cell lymphoma (HS TCL), 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 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 V 1epitope (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 highdose therapy. In conclusion, HS TCL 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. (Blood. 2003;102:4261-4269)