Hepatosplenic T-Cell Lymphoma: Sinusal/Sinusoidal Localization of Malignant Cells Expressing the T-Cell Receptor γδ

• Published in: Blood Vol. 75; no. 11; pp. 2213 - 2219
• Main Authors: Farcet, Jean-Pierre, Gaulard, Philippe, Marolleau, Jean-Pierre, Couedic, Jean-Pierre Le, Henni, Tawfik, Gourdin, Marie-Francoise, Divine, Marine, Haioun, Corinne, Zafrani, Serge, Goossens, Michel, Hercend, Thierry, Reyes, Felix
• Format: Journal Article
• Language: English
• Published: Elsevier Inc 01.06.1990
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood.V75.11.2213.2213

Peripheral T-cell lymphomas consist of a clinically heterogeneous group of malignant disorders whose immunopheno-type 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) δ gene. Lymphoma cells expressed a CD3+ TCR-γδ+ 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-γδ- phenotype with the same δ-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-γδ phenotype of the malignant cells.