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.
Mizuno
Y
Y
Department of Pediatrics, Fukuoka Municipal Hospital for Sick Children and Infectious Disease, Japan.
Morio
T
T
Hara
T
T
D000954:Antigens, Surface / Q000032:analysis; D018450:Disease Progression; D006801:Humans; D007223:Infant; D008218:Lymphocytosis / Q000276:immunology*; D008297:Male; D016176:T-Lymphocyte Subsets / Q000276:immunology / Q000378:metabolism* / Q000648:ultrastructure; D014923:Wiskott-Aldrich Syndrome / Q000276:immunology*
D000954:Antigens, Surface