Autoimmune lymphoproliferative syndrome - Impaired regulation of the immune response by impaired induction of apoptosis

Abstract

Autoimmune lymphoproliferative syndrome (ALPS) is an inherited disease hindering apoptosis of lymphocytes. It includes mutations in genes coding for various components of the apoptotic pathway: CD95 (ALPS 0 and ALPS la), CD178 (ALPS Ib) and caspase-10 (ALPS II). Moreover, patients with genetically inherited caspase-8 or other components of apoptosis-inducing pathway deficiencies (ALPS III) have been described recently. In addition, ALPS-like clinical patterns, including Dianzani's autoimmune lymphoproliferative disease (DALD), were characterized. In this review, we summarize currently known types of ALPS and characterize their immunological and molecular background. © 2006 S. Karger GmbH.