Autoimmune lymphoproliferative syndrome type II

Defects in Caspase 10 are the cause of type II autoimmune lymphoproliferative syndrome (ALPS2). ALPS2 is characterized by abnormal lymphocyte and dendritic cell homeostasis and immune regulatory defects.

Alternative names

ALPS2

Caspase 10 deficiency

Classification

  • Defects in lymphocyte apoptosis
    • Autoimmune lymphoproliferative syndrome

Inheritance

Autosomal dominant

OMIM

#603909 Autoimmune lymphoproliferative syndrome, type II

#601859 Autoimmune lymphoproliferative syndrome, type I

*601762 Caspase 10, apoptosis-related cysteine protease; CASP10

*134637 Tumor necrosis factor receptor superfamily, member 6; TNFRSF6

#605027 non-Hodgkin lymphoma

Incidence

Incidence is not known.