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

作者：Brawura Biskupski Samaha R; Grzela T^*

来源：Transfusion Medicine and Hemotherapy, 2006, 33(1): 71-79.

DOI：10.1159/000090202

• ALPS
• apoptosis
• autoimmune lymphoproliferative syndrome
• caspase
• CD95

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 Ia), 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.

• 出版日期2006