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Autoimmune lymphoproliferative syndrome, type 1b(ALPS1B)

MedGen UID:
Concept ID:
Disease or Syndrome
Synonyms: ALPS1B; Autoimmune Lymphoproliferative Syndrome, Type Ib
Gene (location): FASLG (1q24.3)
OMIM®: 134638; 601859


A rare, primary immunodeficiency with an autosomal dominant pattern of inheritance but incomplete penetrance. It is caused by a mutation in the FASLG (Fas ligand) gene that leads to defective Fas-induced apoptosis. Disruption of Fas-induced apoptosis impairs lymphocyte homeostasis and immune tolerance. Characteristic laboratory findings include an increase in circulating, double-negative (CD4-/CD8-) T cells in the setting of immune-mediated anemia, thrombocytopenia and neutropenia. Clinical signs present in childhood include fatigue, pallor, bruising, hepatosplenomegaly and chronic, non-malignant, non-infectious lymphadenopathy. The clinical course is influenced by a strong association with other autoimmune disorders and an increased risk for developing Hodgkin and non-Hodgkin lymphoma. [from NCI]

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