Hepatosplenic    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 ␥␦ 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 ؉ ,

Epstein-Barr virus–associated lymphoproliferative disorders

“…Because γδ T cells have preferential homing to the sinusoidal areas, marked sinusoidal infiltration is noted in both the liver and spleen [104]. EBV has been reported to be not associated with hepatosplenic T-cell lymphoma in most studies [103,105,106]; however, few studies have reported the detection of EBV genome by Southern blot and in situ hybridization in patients with the disease [85,107]. A hypothesized possible role for EBV in the pathogenesis of the disease can be debated because of the inconsistent EBV detection, although one may argue that it may play a role via chronic antigen exposure [85].…”

Post‐transplant hepatosplenic T‐cell lymphoma successfully treated with hyperCVAD regimen

“…Chronic antigenic stimulation from transplanted allograft and the associated expansion of CD3 1 CD4 2 CD8 2 TCRab 2 T-cells may contribute to the pathogenesis [18]. This hypothesis is supported by reports of HSTL arising in other conditions that are also associated with chronic antigenic stimulation and gd T-cell expansion [3].…”

“…Bone marrow involvement was universal [3]; however, in 6 of 12 cases, the diagnosis was not made on initial bone marrow examination performed to investigate cytopenia. The characteristic sinusoidal lymphoid infiltrate is often subtle and difficult to recognize on routine H&E stain; diagnostic yield can be enhanced by immunohistochemical staining [3,17]. In a large single institution series, the diagnosis of HSTL was initially missed in a third of marrow samples; all were retrospectively found to be involved [3].…”

“…Solid organ transplant recipients account for a significant proportion of HSTL cases: 7 of 45 in a literature review [2] and 4 of 21 in a single institution report [3]. Chronic antigenic stimulation from transplanted allograft and the associated expansion of CD3 1 CD4 2 CD8 2 TCRab 2 T-cells may contribute to the pathogenesis [18].…”

“…Peripheral lymphadenopathy is rare. The disease is clinically aggressive: median survival was 10 months in a literature review of 45 cases [2] and 16 months in a single institution series of 21 cases [3].…”