Immunological analysis of T cells bearing T cell receptor α/β or γ/δ in patients with granular lymphocyte proliferative disorder

Immunological analysis of 2 cases of granular lymphocyte proliferative dis-order with clonal expansion of CD3⁺4-8⁺ T cells bearing either T cell receptor α/β (patient 1) or T cell receptor γ/δ (patient 2) is reported. Significant cyto-toxic activity against K562 was observed in peripheral blood mononuclear cells (PBMC) isolated from patient 2, but not in PBMC from patient 1, although the cytotoxic activity of patient 1 could be augmented following incubation with anti-CD3 monoclonal antibody (MoAb). Northern blot analysis revealed that perforin mRNA was expressed in both patients. Proliferative response to phytohcmagglutinin, anti-CD3 MoAb and 12-0-tetradecanoylphorbol-13-acetate was low in PBMC from both patients compared to that from healthy controls. Cytoplasmic free calcium of PBMC from the 2 patients increased similarly to that of healthy controls after treatment with anti-CD3 MoAb.