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Updated Understanding of Autoimmune Lymphoproliferative Syndrome (ALPS) by Pu Li; Ping Huang; Ye Yang; Mu Hao; Hongwei Peng; Fei Li is a Medicine article available to read on EtoBox.

What is Updated Understanding of Autoimmune Lymphoproliferative Syndrome (ALPS) about?

Autoimmune lymphoproliferative syndrome (ALPS), a disorder characterized by immune dysregulation due to disrupted lymphocyte homeostasis, is mainly resulted from the mutations in FAS-mediated apoptotic pathway. In addition, other mutations of the genes such as Fas-ligand (FASLG), Caspase 10 (CASP10) and Caspase 8 (CASP8), NRAS and KRAS have also been observed in a small number of patients with ALPS or ALPS-related disorders. However, approximately 20-30% of patients with ALPS have unidentified defect. Its clinical manifestations observed in multiple family members include unexplained lymphadenopathy, hepatosplenomegaly, autoimmune cytopenias such as thrombocytopenia, neutropenia, and anemia due to excessive production of antibodies by lymphocytes, elevated number of double-negative T (DNT) cells, and increased risk of lymphoma. As a very rare disease, ALPS was first characterized in the early 1990s. More than 300 families with hereditary ALPS have been reported till now; nearly 500 patients from these families have been studied and followed worldwide over the last 20 years. ALPS has historically considered as a primary immune defect presenting in early childhood, however, recent st

Who reads Updated Understanding of Autoimmune Lymphoproliferative Syndrome (ALPS)?

Author

Pu Li; Ping Huang; Ye Yang; Mu Hao; Hongwei Peng; Fei Li

Publisher

Springer Science and Business Media LLC

Published

2015

Language

EN

Field

Medicine (Health Sciences)