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.

A case of hepatosplenic γ–δ T‐cell lymphoma with a transient response to Fludarabine and Alemtuzumab

“…Farcet et al (12) first described the hepatosplenic γ – δ T‐cell lymphoma in 1990. It was recognized in the Revised European American Lymphoma (REAL) classification in 1994.…”

Lymphoproliferative disease of granular lymphocytes with T‐cell receptor gamma delta‐positive phenotype: restricted usage of T‐cell receptor gamma and delta subunit genes

“…Among leukemias or lymphomas with the T‐cell phenotype, hepatosplenic γ δ T‐cell lymphoma and some forms of subcutaneous panniculitis‐like T‐cell lymphoma (SPTCL) are the only diseases commonly known to feature neoplastic expansion of γ δ T cells (28). Hepatosplenic γ δ T‐cell lymphoma commonly shows V δ 1 and V γ 9 (29, 30) while the usage of TCR γ and δ subunit genes in SPTCL has not yet been examined (31). However, hepatosplenic γ δ T‐cell lymphoma follows rather aggressive clinical courses, requires multi‐drug chemotherapy and is usually found in much younger people (32).…”

Treatment and prognosis of mature (non‐anaplastic) T‐ and NK‐cell lymphomas in childhood, adolescents, and young adults

“…In fact, the first report to suggest the existence of a new PTCL entity described two young adults with the typical presentation of massive liver and spleen enlargement, and thrombocytopenia (Farcet et al , ). The authors described the presence of peculiar marrow, hepatic and splenic sinusal/sinusoidal infiltration that is now considered the hallmark of this disease (Farcet et al , ). Lymphoma cells are usually of γδ T‐cell origin and are CD3+, TCRδ1+, TCRα/β−, CD56+/−, CD4−, CD8−/+ and CD5−.…”