Hepatosplenic T-cell lymphoma: sinusal/sinusoidal localization of malignant cells expressing the T-cell receptor gamma delta.
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.
Farcet
J P
JP
INSERM U91, Département d'Enseignement, Hôpital Henri Mondor, Créteil, France.
Gaulard
P
P
Marolleau
J P
JP
Le Couedic
J P
JP
Henni
T
T
Gourdin
M F
MF
Divine
M
M
Haioun
C
C
Zafrani
S
S
Goossens
M
M
D000328:Adult; D015870:Gene Expression; D005838:Genotype; D006801:Humans; D008113:Liver Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000648:ultrastructure; D008223:Lymphoma / Q000378:metabolism / Q000473:pathology* / Q000648:ultrastructure; D010641:Phenotype; D011948:Receptors, Antigen, T-Cell / Q000235:genetics / Q000378:metabolism*; D016692:Receptors, Antigen, T-Cell, gamma-delta; D013160:Splenic Neoplasms / Q000378:metabolism / Q000473:pathology* / Q000648:ultrastructure; D013601:T-Lymphocytes / Q000378:metabolism / Q000648:ultrastructure*
D011948:Receptors, Antigen, T-Cell; D016692:Receptors, Antigen, T-Cell, gamma-delta