Peripheral expansion of V delta 1-J delta 1/J delta 2+ gamma delta T cells and large granular lymphocytes in a patient with Wiskott-Aldrich syndrome.

A 7 month old Japanese boy was diagnosed to have Wiskott-Aldrich syndrome (WAS) because of eczema, thrombocytopenia, progressive immune defect and CD43 (sialophorin) abnormality. He had developed repeated infections since 16 months of age. Gamma delta T cell-receptor positive T cells in the peripheral blood were gradually increased from 3.1% (7 months of age) to 5.6% (12 months), 19.6% (18 months) and 56.7% (25 months). The phenotypes of expanded gamma delta T cells were delta TCS1-positive (V delta 1-J delta 1/J delta 2) and CD8 dim-positive. The proportion of increased granular lymphocytes correlated well with that of gamma delta T cells. The significance of peripheral expansion of gamma delta T cells and granular lymphocytes in WAS is discussed.