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Peripheral expansion of Vδ1‐Jδ1/Jδ2 + γδT cells and large granular lymphocytes in a patient with Wiskott‐Aldrich syndrome
Author(s) -
MIZUNO YUMI,
MORIO TOMOHIRO,
HARA TOSHIRO
Publication year - 1995
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/j.1442-200x.1995.tb03340.x
Subject(s) - medicine , wiskott–aldrich syndrome , peripheral , peripheral blood , cd8 , cd43 , immune system , abnormality , immunology , pathology , antibody , genetics , cd20 , psychiatry , gene , biology
Abstract 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. γδT 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 γδT cells were δTCS1‐positive (Vδ1‐Jδ1/Jδ2) and CD8 dim‐positive. The proportion of increased granular lymphocytes correlated well with that of γδT cells. The significance of peripheral expansion of γδT cells and granular lymphocytes in WAS is discussed.