TcR-α/β+ CD4− CD8− T cells in humans with the autoimmune lymphoproliferative syndrome express a novel CD45 isoform that is analogous to murine B220 and …
JJH Bleesing, MR Brown, JK Dale, SE Straus… - Clinical …, 2001 - Elsevier
Autoimmune lymphoproliferative syndrome (ALPS), caused by inherited defects in apoptosis
secondary to mutations in genes encoding Fas/CD95/APO-1 and Fas ligand (Fasl)/CD95L,
is characterized by nonmalignant lymphadenopathy and splenomegaly, increased T cell
receptor α/β+ CD4− CD8− T cells (α/β+ double-negative T cells [α/β+-DNT cells]),
autoimmunity, hypergammaglobulinemia, and cytokine abnormalities. The α/β+-DNT cells
are immunophenotypically and functionally similar to α/β+-DNT cells that accumulate in lpr …
secondary to mutations in genes encoding Fas/CD95/APO-1 and Fas ligand (Fasl)/CD95L,
is characterized by nonmalignant lymphadenopathy and splenomegaly, increased T cell
receptor α/β+ CD4− CD8− T cells (α/β+ double-negative T cells [α/β+-DNT cells]),
autoimmunity, hypergammaglobulinemia, and cytokine abnormalities. The α/β+-DNT cells
are immunophenotypically and functionally similar to α/β+-DNT cells that accumulate in lpr …