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Classically, ALPS is due to mutations in<jats:italic>FAS<\/jats:italic>and other related genes; however, recent research revealed that other genes could be responsible for similar clinical features. Therefore, ALPS classification and diagnostic criteria have changed over time, and several ALPS-like disorders have been recently identified. Moreover, mutations in<jats:italic>FAS<\/jats:italic>often show an incomplete penetrance, and certain genotypes have been associated to a dominant or recessive inheritance pattern.<jats:italic>FAS<\/jats:italic>mutations may also be acquired or could become pathogenic when associated to variants in other genes, delineating a possible digenic type of inheritance. Intriguingly, variants in<jats:italic>FAS<\/jats:italic>and increased TCR \u03b1\u03b2 double-negative T cells (DNTs, a hallmark of ALPS) have been identified in multifactorial autoimmune diseases, while FAS itself could play a potential role in carcinogenesis. These findings suggest that alterations of FAS-mediated apoptosis could trespass the universe of inborn errors of immunity and that somatic mutations leading to ALPS could only be the tip of the iceberg of acquired immunodeficiencies.<\/jats:p>","DOI":"10.1007\/s00277-022-04761-7","type":"journal-article","created":{"date-parts":[[2022,1,20]],"date-time":"2022-01-20T21:11:05Z","timestamp":1642713065000},"page":"469-484","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":46,"title":["ALPS, FAS, and beyond: from inborn errors of immunity to acquired immunodeficiencies"],"prefix":"10.1007","volume":"101","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6879-8982","authenticated-orcid":false,"given":"Filippo","family":"Consonni","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4676-6541","authenticated-orcid":false,"given":"Eleonora","family":"Gambineri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudio","family":"Favre","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2022,1,20]]},"reference":[{"key":"4761_CR1","first-page":"295","volume":"2017","author":"C Speckmann","year":"2017","unstructured":"Speckmann C, Borkhardt A, Gaspar HB et al (2017) Genetic disorders of immune regulation. 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