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B Falini, L Flenghi, M Fagioli, MF Martelli, S Pileri, F Grignani, A Beltrami, D Novero and PG Pelicci
Institute of Clinical Medicine, University of Perugia, Italy.
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.
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