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Hepatosplenic T-cell lymphoma: sinusal/sinusoidal localization of malignant
cells expressing the T-cell receptor gamma delta
JP Farcet, P Gaulard, JP Marolleau, JP Le Couedic, T Henni, MF Gourdin, M Divine, C Haioun, S Zafrani and M Goossens
INSERM U91, Departement d'Enseignement, Hopital Henri Mondor, Creteil,
France.
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.
Volume 75,
Issue 11,
pp. 2213-2219,
06/01/1990
Copyright © 1990 by The American Society of Hematology
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