{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T20:43:05Z","timestamp":1791405785152,"version":"4.3.3"},"reference-count":53,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2021,5,27]],"date-time":"2021-05-27T00:00:00Z","timestamp":1622073600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Allergy Immunology"],"published-print":{"date-parts":[[2021,10]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Autoimmune lymphoproliferative syndrome (ALPS) is a group of genetic disorders characterized by early\u2010onset lymphoproliferation, autoimmune cytopenias, and susceptibility to lymphoma. The majority of ALPS patients carry heterozygous germline mutations in the TNFRSF6 gene. In this study, we conducted a systematic review of patients with ALPS and ALPS\u2010like syndrome.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>The literature search was performed in Web of Science, Scopus, and PubMed databases to find eligible studies. Additionally, the reference list of all included papers was hand\u2010searched for additional studies. Demographic, clinical, immunological, and molecular data were extracted and compared between the ALPS and ALPS\u2010like syndrome.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Totally, 720 patients with ALPS (532 genetically determined and 189 genetically undetermined ALPS) and 59 cases with ALPS\u2010like phenotype due to mutations in genes other than ALPS genes were assessed. In both ALPS and ALPS\u2010like patients, splenomegaly was the most common clinical presentation followed by autoimmune cytopenias and lymphadenopathy. Among other clinical manifestations, respiratory tract infections were significantly higher in ALPS\u2010like patients than ALPS. The immunological analysis showed a lower serum level of IgA, IgG, and lymphocyte count in ALPS\u2010like patients compared to ALPS. Most (85%) of the ALPS and ALPS\u2010like cases with determined genetic defects carry mutations in the FAS gene. About one\u2010third of patients received immunosuppressive therapy with conventional or targeted immunotherapy agents. A small fraction of patients (3.3%) received hematopoietic stem cell transplantation with successful engraftment, and all except two patients survived after transplantation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Our results showed that the FAS gene with 85% frequency is the main etiological cause of genetically diagnosed patients with ALPS phenotype; therefore, the genetic defect of the majority of suspected ALPS patients could be confirmed by mutation analysis of FAS gene.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/pai.13535","type":"journal-article","created":{"date-parts":[[2021,5,9]],"date-time":"2021-05-09T02:27:17Z","timestamp":1620527237000},"page":"1519-1532","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":36,"title":["Clinical, immunological, and genetic features in 780 patients with autoimmune lymphoproliferative syndrome (ALPS) and ALPS\u2010like diseases: A systematic review"],"prefix":"10.1111","volume":"32","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3666-1620","authenticated-orcid":false,"given":"Nasim","family":"Hafezi","sequence":"first","affiliation":[{"name":"Department of Immunology School of Medicine Mazandaran University of Medical Sciences  Sari Iran"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1642-3289","authenticated-orcid":false,"given":"Majid","family":"Zaki\u2010Dizaji","sequence":"additional","affiliation":[{"name":"Legal Medicine Research Center Legal Medicine Organization  Tehran Iran"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7092-4239","authenticated-orcid":false,"given":"Matineh","family":"Nirouei","sequence":"additional","affiliation":[{"name":"Student Research Committee Alborz University of Medical Sciences  Karaj 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