Immunological Analysis of T Cells Bearing T Cell Receptor α/β or γ/δ in Patients with Granular Lymphocyte Proliferative Disorder

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Abstract
Immunological analysis of 2 cases of granular lymphocyte proliferative disorder with clonal expansion of CD3+4-8+ T cells bearing either T cell receptor alpha/beta (patient 1) or T cell receptor gamma/delta (patient 2) is reported. Significant cytotoxic 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 phytohemagglutinin, anti-CD3 MoAb and 12-O-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.

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