Hepatosplenic T-cell lymphoma: sinusal/sinusoidal localization of malignant cells expressing the T-cell receptor gamma delta

Abstract

Peripheral T-cell lymphomas consist of a clinically heterogeneous group of malignant disorders whose immunophenotype usually corresponds to that of normal mature T cells. We describe and correlate the clinical, histopathologic, phenotypic, and genotypic findings in two patients with malignant lymphoma presenting with hepatosplenic disease. The morphologic pattern of lymphoma was that of a sinusal/sinusoidal infiltration in spleen, marrow, and liver. This morphologic characteristic was associated with the presence of a productive clonal rearrangement of the T-cell receptor (TCR) delta gene. Lymphoma cells expressed a CD3-TCR-gamma delta- phenotype. They were also double negative (ie, CD4-CD8-) and lacked the CD5 and CD7 antigens. In one patient, tumor progression was associated with phenotypic changes that resulted in a CD3-TCR-gamma delta- phenotype with the same delta-gene rearrangement as initially. These observations suggest the existence of a new type of peripheral T-cell lymphoma characterized by its hepatosplenic presentation, and by the sinusal/sinusoidal tropism and the TCR-gamma delta phenotype of the malignant cells.

Hepatosplenic γδ T‐cell lymphoma: relation to Epstein–Barr virus and activated cytotoxic molecules

“…The majority of previously reported patients have been young adults with a striking male predominance. [3][4][5] However, one of our patients was significantly older, and two elderly cases have also been reported elsewhere. 6 Hepatosplenic ␥␦ TCLs express a characteristic 'double-negative' T-cell phenotype (CD4¹, CD8¹, CD3þ, TCR␥␦þ).…”

“…Histological examination showed pleomorphic medium-and large-sized cells in all three cases. Hepatosplenic ␥␦ TCL is a rare, clinically aggressive subtype of peripheral T-cell lymphoma (PCTL), [3][4][5][6] that has recently been proposed as a provisional category within the Revised European-American Classification of lymphoid neoplasms. 17 Most patients present with hepatosplenomegaly, clinically significant cytopenia and B-symptoms without significant lymphadenopathy.…”

“…17 Most patients present with hepatosplenomegaly, clinically significant cytopenia and B-symptoms without significant lymphadenopathy. [3][4][5][6] Lymphoma preferentially involves hepatic sinusoids, splenic red pulp and bone marrow. Cytologically, the cells are medium in size, and monomorphic in appearance.…”

“…1,2 Most postthymic neoplasms that arise from this ␥␦ T-cell subpopulation can be classified as hepatosplenic ␥␦ lymphomas. [3][4][5][6] On the other hand, only a few cases of non-hepatosplenic ␥␦ lymphoma have been reported. 7,8 Hepatosplenic ␥␦ T-cell lymphoma is a rare, recently described subset of peripheral T-cell lymphomas characterized by hepatosplenomegaly without lymphadenopathy, clinically significant cytopenia and an aggressive clinical course.…”

“…7,8 Hepatosplenic ␥␦ T-cell lymphoma is a rare, recently described subset of peripheral T-cell lymphomas characterized by hepatosplenomegaly without lymphadenopathy, clinically significant cytopenia and an aggressive clinical course. [3][4][5][6] These lymphomas preferentially infiltrate hepatic sinusoids and splenic red pulp but may also involve lymph node sinuses, bone marrow and peripheral blood. 5,6 Non-hepatosplenic ␥␦ T-cell lymphoma can be regarded as a model of lymphoma derived from activated cytotoxic cells, occurring in mucosa or skin.…”