T-lymphoblastic lymphomas expressing the non-disulfide-linked form of the T-cell receptor gamma/delta: characterization with monoclonal antibodies and genotypic analysis.
Three cases of T-lymphoblastic lymphomas (T-LL) expressing the T cell antigen receptor gamma delta (TCR gamma delta) are reported. All of them were CD3+/beta F1-/TCR delta 1+. Moreover, neoplastic cells reacted with the delta TCS1 monoclonal antibody (MoAb) which binds to the non-disulfide-linked form of the TCR gamma delta, but not with the BB3 MoAb which recognizes the disulfide-linked form of the TCR gamma delta. All cases showed a stage II cortical phenotype, eg, TdT+/CD1+/CD3+/CD5+/CD7+; two of them coexpressed CD4/CD8, while the other was CD4+/CD8-. Two cases were positive for CALLA and CD25. Immunogenotypic analysis showed evidence of T beta and C gamma 2 gene rearrangements in all three cases and immunoglobulin (Ig) gene rearrangements in two cases. Two patients presented with an anterior mediastinal mass and the third with a solitary inguinal lymphadenopathy. We suggest that these cases of TCR gamma delta+ T-LL may be derived from the small population (approximately 0.5%) of CD3+ cortical thymocytes which, in the normal human thymus, express the delta TCS1-reactive, non-disulfide-linked form of the TCR gamma delta.
Falini
B
B
Institute of Clinical Medicine, University of Perugia, Italy.
Flenghi
L
L
Fagioli
M
M
Martelli
M F
MF
Pileri
S
S
Grignani
F
F
Beltrami
A
A
Novero
D
D
Pelicci
P G
PG
D000911:Antibodies, Monoclonal / Q000276:immunology; D015139:Blotting, Southern; D015326:Gene Rearrangement, B-Lymphocyte, Heavy Chain; D015329:Gene Rearrangement, T-Lymphocyte; D006801:Humans; D008198:Lymph Nodes / Q000276:immunology; D008223:Lymphoma / Q000235:genetics / Q000276:immunology / Q000503:physiopathology*; D011948:Receptors, Antigen, T-Cell / Q000502:physiology*; D016693:Receptors, Antigen, T-Cell, alpha-beta; D016692:Receptors, Antigen, T-Cell, gamma-delta; D013601:T-Lymphocytes / Q000502:physiology*
D000911:Antibodies, Monoclonal; D011948:Receptors, Antigen, T-Cell; D016693:Receptors, Antigen, T-Cell, alpha-beta; D016692:Receptors, Antigen, T-Cell, gamma-delta