A composite picture of TcRα/β+ CD4− CD8− T Cells (α/β-DNTCs) in humans with autoimmune lymphoproliferative syndrome

JJH Bleesing, MR Brown, C Novicio, D Guarraia… - Clinical …, 2002 - Elsevier
The discovery of an unusual T-cell subset characterized by the expression of the α/β T-cell
receptor without expression of either CD4 or CD8 [α/β-double-negative T cells (α/β-DNTCs)]
provided critical insights in the evaluation of a “new” lymphoproliferative disorder known as
autoimmune lymphoproliferative syndrome (ALPS). ALPS is a disorder of defective Fas-
mediated lymphocyte apoptosis, manifested by accumulation of α/β-DNTCs and other
lymphocyte subsets, leading to lymphadenopathy and splenomegaly, autoimmunity, and an …