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.