{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T22:31:20Z","timestamp":1790289080218,"version":"4.1.0"},"reference-count":24,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2018,10,16]],"date-time":"2018-10-16T00:00:00Z","timestamp":1539648000000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100009400","name":"Medizinische Fakult\u00e4t, Heinrich-Heine-Universit\u00e4t D\u00fcsseldorf","doi-asserted-by":"publisher","award":["2016-70"],"award-info":[{"award-number":["2016-70"]}],"id":[{"id":"10.13039\/501100009400","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"DOI":"10.3389\/fimmu.2018.02400","type":"journal-article","created":{"date-parts":[[2018,10,16]],"date-time":"2018-10-16T08:55:53Z","timestamp":1539680153000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":52,"title":["EBV Negative Lymphoma and Autoimmune Lymphoproliferative Syndrome Like Phenotype Extend the Clinical Spectrum of Primary Immunodeficiency Caused by STK4 Deficiency"],"prefix":"10.3389","volume":"9","author":[{"given":"Cyrill","family":"Schipp","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Schl\u00fctermann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"H\u00f6nscheid","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Schafiq","family":"Nabhani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jessica","family":"H\u00f6ll","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Prasad T.","family":"Oommen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sebastian","family":"Ginzel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bernhard","family":"Fleckenstein","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bj\u00f6rn","family":"Stork","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arndt","family":"Borkhardt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Polina","family":"Stepensky","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ute","family":"Fischer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2018,10,16]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"3450","DOI":"10.1182\/blood-2011-09-378158","article-title":"The phenotype of human STK4 deficiency","volume":"119","author":"Abdollahpour","year":"2012","journal-title":"Blood"},{"key":"B2","doi-asserted-by":"publisher","first-page":"3458","DOI":"10.1182\/blood-2011-09-378364","article-title":"MST1 mutations in autosomal recessive primary immunodeficiency characterized by defective naive T-cell survival","volume":"119","author":"Nehme","year":"2012","journal-title":"Blood"},{"key":"B3","doi-asserted-by":"publisher","first-page":"e44010","DOI":"10.1371\/journal.pone.0044010","article-title":"Inherited MST1 deficiency underlies susceptibility to EV-HPV infections","volume":"7","author":"Crequer","year":"2012","journal-title":"PLoS ONE"},{"key":"B4","doi-asserted-by":"publisher","first-page":"316","DOI":"10.1016\/j.clim.2015.06.010","article-title":"STK4 (MST1) deficiency in two siblings with autoimmune cytopenias: a novel mutation","volume":"161","author":"Halacli","year":"2015","journal-title":"Clin Immunol."},{"key":"B5","doi-asserted-by":"publisher","first-page":"117","DOI":"10.1007\/s10875-016-0232-2","article-title":"Defective leukocyte adhesion and chemotaxis contributes to combined immunodeficiency in humans with autosomal recessive MST1 deficiency","volume":"36","author":"Dang","year":"2016","journal-title":"J Clin Immunol."},{"key":"B6","doi-asserted-by":"publisher","first-page":"e8852","DOI":"10.1097\/MD.0000000000008852","article-title":"EBV lymphoproliferative-associated disease and primary cardiac T-cell lymphoma in a STK4 deficient patient: a case report","volume":"96","author":"Sherkat","year":"2017","journal-title":"Medicine"},{"key":"B7","doi-asserted-by":"publisher","first-page":"1350","DOI":"10.1172\/JCI37901","article-title":"Girls homozygous for an IL-2-inducible T cell kinase mutation that leads to protein deficiency develop fatal EBV-associated lymphoproliferation","volume":"119","author":"Huck","year":"2009","journal-title":"J Clin Invest."},{"key":"B8","doi-asserted-by":"publisher","first-page":"S109","DOI":"10.1684\/mrh.2011.0286","article-title":"Loss of MAGT1 abrogates the Mg2+ flux required for T cell signaling and leads to a novel human primary immunodeficiency","volume":"24","author":"Li","year":"2011","journal-title":"Magnes Res."},{"key":"B9","doi-asserted-by":"publisher","first-page":"963","DOI":"10.1038\/leu.2011.371","article-title":"Loss-of-function mutations within the IL-2 inducible kinase ITK in patients with EBV-associated lymphoproliferative diseases","volume":"26","author":"Linka","year":"2012","journal-title":"Leukemia"},{"key":"B10","doi-asserted-by":"publisher","first-page":"684","DOI":"10.1007\/s10875-016-0317-y","article-title":"Fatal lymphoproliferative disease in two siblings lacking functional FAAP24","volume":"36","author":"Daschkey","year":"2016","journal-title":"J Clin Immunol."},{"key":"B11","doi-asserted-by":"publisher","first-page":"935","DOI":"10.1016\/S0140-6736(75)92004-8","article-title":"X-linked recessive progressive combined variable immunodeficiency (Duncan's disease)","volume":"1","author":"Purtilo","year":"1975","journal-title":"Lancet"},{"key":"B12","doi-asserted-by":"publisher","first-page":"110","DOI":"10.1038\/nature05257","article-title":"XIAP deficiency in humans causes an X-linked lymphoproliferative syndrome","volume":"444","author":"Rigaud","year":"2006","journal-title":"Nature"},{"key":"B13","doi-asserted-by":"publisher","first-page":"473","DOI":"10.3324\/haematol.2012.068791","article-title":"Combined immunodeficiency with life-threatening EBV-associated lymphoproliferative disorder in patients lacking functional CD27","volume":"98","author":"Salzer","year":"2013","journal-title":"Haematologica"},{"key":"B14","doi-asserted-by":"publisher","first-page":"787","DOI":"10.1016\/j.jaci.2011.11.013","article-title":"CD27 deficiency is associated with combined immunodeficiency and persistent symptomatic EBV viremia","volume":"129","author":"van","year":"2012","journal-title":"J Allergy Clin Immunol."},{"key":"B15","doi-asserted-by":"publisher","first-page":"5386","DOI":"10.1158\/0008-5472.CAN-11-3956","article-title":"Mammalian sterile 20-like kinase 1 suppresses lymphoma development by promoting faithful chromosome segregation","volume":"72","author":"Kim","year":"2012","journal-title":"Cancer Res."},{"key":"B16","doi-asserted-by":"publisher","first-page":"55","DOI":"10.1016\/0167-5699(95)80087-5","article-title":"Immortalization of human T cells by Herpesvirus saimiri","volume":"16","author":"Meinl","year":"1995","journal-title":"Immunology Today"},{"key":"B17","doi-asserted-by":"publisher","first-page":"1754","DOI":"10.1093\/bioinformatics\/btp324","article-title":"Fast and accurate short read alignment with Burrows-Wheeler transform","volume":"25","author":"Li","year":"2009","journal-title":"Bioinformatics"},{"key":"B18","doi-asserted-by":"publisher","first-page":"2078","DOI":"10.1093\/bioinformatics\/btp352","article-title":"The sequence alignment\/map format and SAMtools","volume":"25","author":"Li","year":"2009","journal-title":"Bioinformatics"},{"key":"B19","doi-asserted-by":"publisher","first-page":"e35","DOI":"10.1182\/blood-2010-04-280347","article-title":"Revised diagnostic criteria and classification for the autoimmune lymphoproliferative syndrome (ALPS): report from the 2009 NIH International Workshop","volume":"116","author":"Oliveira","year":"2010","journal-title":"Blood"},{"key":"B20","doi-asserted-by":"publisher","first-page":"20160271","DOI":"10.1098\/rstb.2016.0271","article-title":"Epstein-Barr virus-associated lymphomas","volume":"372","author":"Shannon-Lowe","year":"2017","journal-title":"Phil Trans R Soc B."},{"key":"B21","doi-asserted-by":"publisher","first-page":"E1312","DOI":"10.1073\/pnas.1110428108","article-title":"Mst1 and Mst2 protein kinases restrain intestinal stem cell proliferation and colonic tumorigenesis by inhibition of Yes-associated protein (Yap) overabundance","volume":"108","author":"Zhou","year":"2011","journal-title":"Proc Natl Acad Sci USA"},{"key":"B22","doi-asserted-by":"publisher","first-page":"425","DOI":"10.1016\/j.ccr.2009.09.026","article-title":"Mst1 and Mst2 maintain hepatocyte quiescence and suppress hepatocellular carcinoma development through inactivation of the Yap1 oncogene","volume":"16","author":"Zhou","year":"2009","journal-title":"Cancer Cell."},{"key":"B23","doi-asserted-by":"publisher","first-page":"37","DOI":"10.4103\/2231-0770.114121","article-title":"Idiopathic CD4 lymphocytopenia: spectrum of opportunistic infections, malignancies, and autoimmune diseases","volume":"3","author":"Ahmad","year":"2013","journal-title":"Avicenna J Med."},{"key":"B24","doi-asserted-by":"publisher","first-page":"1365","DOI":"10.1136\/jcp.2007.051953","article-title":"HIV infection and lymphoma","volume":"60","author":"Grogg","year":"2007","journal-title":"J Clin Pathol."}],"container-title":["Frontiers in Immunology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/article\/10.3389\/fimmu.2018.02400\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2018,10,16]],"date-time":"2018-10-16T08:55:56Z","timestamp":1539680156000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/article\/10.3389\/fimmu.2018.02400\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,10,16]]},"references-count":24,"alternative-id":["10.3389\/fimmu.2018.02400"],"URL":"https:\/\/doi.org\/10.3389\/fimmu.2018.02400","relation":{},"ISSN":["1664-3224"],"issn-type":[{"value":"1664-3224","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,10,16]]},"article-number":"2400"}}