HEPATOSPLENIC T-CELL LYMPHOMA - SINUSAL SINUSOIDAL LOCALIZATION OF MALIGNANT-CELLS EXPRESSING THE T-CELL RECEPTOR-GAMMA-DELTA

  • 1 June 1990
    • journal article
    • research article
    • Vol. 75  (11) , 2213-2219
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.