Lymphoproliferative disease of granular lymphocytes with T-cell receptor gamma delta-positive phenotype: restricted usage of T-cell receptor gamma and delta subunit genes

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Abstract
Lymphoproliferative disease of granular lymphocytes (LDGL) is characterized by more than 0.5 × 109/L of proliferating granular lymphocytes in the peripheral blood. Because of its rarity, the characteristics of LDGL with T‐cell receptor (TCR) γδ phenotype (γδT‐LDGL) have not yet been identified. This report describes the clinical, hematological, and immunological findings of four patients with this disease. In two cases, the clinical course was indolent and the other two patients required various therapies. The cells had a common immunophenotype: CD3+, CD4–, CD16+, CD56–, CD57–, CD122–, TCR‐γδ+, and three were CD8‐positive. The immunopurified TCR‐γδ cells from the patients expressed only Vγ9 and Vδ1. Spectratyping and sequencing showed mono‐ or oligoclonality for TCRγ and TCRδ subunit genes. Soluble Fas ligand in sera was significantly elevated in all patients. These findings suggest that γδT‐LDGL qualifies as a distinct disease entity.

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