Article
Clinical and immunological overlap between autoimmune lymphoproliferative syndrome and common variable immunodeficiency.
Clinical immunology (Orlando, Fla.) - 1 Dec 2010
Rensing-Ehl A, Warnatz K, Fuchs S, Schlesier M, Salzer U, Draeger R, Bondzio I, Joos Y, Janda A, Gomes M, Abinun M, Hambleton S, Cant A, Shackley F, Flood T, Waruiru C, Beutel K, Siepermann K, Dueckers G, Niehues T, Wiesel T, Schuster V, Seidel M G, Minkov M, Sirkiä K, Kopp M V, Korhonen M, Schwarz K, Ehl S, Speckmann C
Abstract excerpt
Autoimmune lymphoproliferative syndrome (ALPS) is mainly caused by defects in the CD95 pathway. Raised CD3+TCRαβ+CD4-CD8- double negative T cells and impaired T cell apoptosis are hallmarks of the disease. In contrast, the B cell compartment has been less well studied. We found an altered distrib...
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