{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T21:55:44Z","timestamp":1790286944126,"version":"4.1.0"},"publisher-location":"Berlin, Heidelberg","reference-count":260,"publisher":"Springer Berlin Heidelberg","content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025]]},"DOI":"10.1007\/82_2025_316","type":"book-chapter","created":{"date-parts":[[2025,8,12]],"date-time":"2025-08-12T15:17:54Z","timestamp":1755011874000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Primary Immunodeficiencies and EBV-Associated Diseases"],"prefix":"10.1007","author":[{"given":"Benjamin","family":"Fournier","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sylvain","family":"Latour","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,8,13]]},"reference":[{"key":"316_CR1","doi-asserted-by":"crossref","first-page":"3450","DOI":"10.1182\/blood-2011-09-378158","volume":"119","author":"H Abdollahpour","year":"2012","unstructured":"Abdollahpour H et al (2012) The phenotype of human STK4 deficiency. Blood 119:3450\u20133457","journal-title":"Blood"},{"key":"316_CR2","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1084\/jem.20160849","volume":"214","author":"H Abolhassani","year":"2017","unstructured":"Abolhassani H et al (2017) Combined immunodeficiency and Epstein-Barr virus-induced B cell malignancy in humans with inherited CD70 deficiency. J Exp Med 214:91\u2013106","journal-title":"J Exp Med"},{"key":"316_CR3","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1007\/s10875-015-0141-9","volume":"35","author":"C Aguilar","year":"2015","unstructured":"Aguilar C, Latour S (2015) X-linked inhibitor of apoptosis protein deficiency: more than an X-linked lymphoproliferative syndrome. J Clin Immunol 35:331\u2013338","journal-title":"J Clin Immunol"},{"key":"316_CR4","doi-asserted-by":"crossref","first-page":"1131","DOI":"10.1016\/j.jaci.2014.04.031","volume":"134","author":"C Aguilar","year":"2014","unstructured":"Aguilar C et al (2014) Characterization of Crohn disease in X-linked inhibitor of apoptosis-deficient male patients and female symptomatic carriers. J Allergy Clin Immunol 134:1131\u20131141 e1139","journal-title":"J Allergy Clin Immunol"},{"key":"316_CR5","doi-asserted-by":"crossref","first-page":"13353","DOI":"10.1074\/jbc.273.21.13353","volume":"273","author":"H Akiba","year":"1998","unstructured":"Akiba H et al (1998) CD27, a member of the tumor necrosis factor receptor superfamily, activates NF-kappaB and stress-activated protein kinase\/c-Jun N-terminal kinase via TRAF2, TRAF5, and NF-kappaB-inducing kinase. J Biol Chem 273:13353\u201313358","journal-title":"J Biol Chem"},{"key":"316_CR6","doi-asserted-by":"crossref","first-page":"1720","DOI":"10.1038\/s41375-022-01620-2","volume":"36","author":"R Alaggio","year":"2022","unstructured":"Alaggio R et al (2022) The 5th edition of the World Health Organization classification of haematolymphoid tumours: lymphoid neoplasms. Leukemia 36:1720\u20131748","journal-title":"Leukemia"},{"key":"316_CR7","doi-asserted-by":"crossref","first-page":"1944","DOI":"10.1038\/s41375-023-01962-5","volume":"37","author":"R Alaggio","year":"2023","unstructured":"Alaggio R et al (2023) Correction: \"the 5th edition of the World Health Organization classification of haematolymphoid tumours: lymphoid neoplasms\" leukemia. 2022 Jul;36(7):1720-1748. Leukemia 37:1944\u20131951","journal-title":"Leukemia"},{"key":"316_CR8","doi-asserted-by":"crossref","first-page":"703","DOI":"10.1016\/j.jaci.2015.02.022","volume":"136","author":"OK Alkhairy","year":"2015","unstructured":"Alkhairy OK et al (2015) Novel mutations in TNFRSF7\/CD27: clinical, immunologic, and genetic characterization of human CD27 deficiency. J Allergy Clin Immunol 136:703\u2013712 e710","journal-title":"J Allergy Clin Immunol"},{"key":"316_CR9","doi-asserted-by":"crossref","first-page":"507","DOI":"10.1097\/MPH.0000000000000366","volume":"37","author":"C Alme","year":"2015","unstructured":"Alme C, Satwani P, Alobeid B, Bhagat G, Kelly KM (2015) Atypical clinical course in pediatric Hodgkin lymphoma: association with germline mutations in Interleukin-2-inducible T-cell kinase. J Pediatr Hematol Oncol 37:507\u2013508","journal-title":"J Pediatr Hematol Oncol"},{"key":"316_CR10","doi-asserted-by":"crossref","first-page":"574","DOI":"10.1016\/j.jaci.2019.03.002","volume":"144","author":"MF Alosaimi","year":"2019","unstructured":"Alosaimi MF et al (2019) Immunodeficiency and EBV-induced lymphoproliferation caused by 4-1BB deficiency. J Allergy Clin Immunol 144:574\u2013583 e575","journal-title":"J Allergy Clin Immunol"},{"key":"316_CR11","doi-asserted-by":"crossref","first-page":"744","DOI":"10.1182\/blood.V85.3.744.bloodjournal853744","volume":"85","author":"I Anagnostopoulos","year":"1995","unstructured":"Anagnostopoulos I, Hummel M, Kreschel C, Stein H (1995) Morphology, immunophenotype, and distribution of latently and\/or productively Epstein-Barr virus-infected cells in acute infectious mononucleosis: implications for the interindividual infection route of Epstein-Barr virus. Blood 85:744\u2013750","journal-title":"Blood"},{"key":"316_CR12","doi-asserted-by":"crossref","DOI":"10.1101\/cshperspect.a002287","volume":"2","author":"AH Andreotti","year":"2010","unstructured":"Andreotti AH, Schwartzberg PL, Joseph RE, Berg LJ (2010) T-cell signaling regulated by the Tec family kinase, Itk. Cold Spring Harb Perspect Biol 2:a002287","journal-title":"Cold Spring Harb Perspect Biol"},{"key":"316_CR13","doi-asserted-by":"crossref","first-page":"14","DOI":"10.3389\/fped.2019.00014","volume":"7","author":"A Arai","year":"2019","unstructured":"Arai A (2019) Advances in the study of chronic active Epstein-Barr virus infection: clinical features under the 2016 WHO classification and mechanisms of development. Front Pediatr 7:14","journal-title":"Front Pediatr"},{"key":"316_CR14","doi-asserted-by":"crossref","DOI":"10.1097\/MD.0000000000018601","volume":"99","author":"F Ashrafi","year":"2020","unstructured":"Ashrafi F, Klein C, Poorpooneh M, Sherkat R, Khoshnevisan R (2020) A case report of sinusoidal diffuse large B-cell lymphoma in a STK4 deficient patient. Medicine (Baltimore) 99:e18601","journal-title":"Medicine (Baltimore)"},{"key":"316_CR15","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1007\/s10875-014-0126-0","volume":"35","author":"SE Aydin","year":"2015","unstructured":"Aydin SE et al (2015) DOCK8 deficiency: clinical and immunological phenotype and treatment options - a review of 136 patients. J Clin Immunol 35:189\u2013198","journal-title":"J Clin Immunol"},{"key":"316_CR16","doi-asserted-by":"crossref","first-page":"363","DOI":"10.1056\/NEJM197402142900704","volume":"290","author":"RS Bar","year":"1974","unstructured":"Bar RS et al (1974) Fatal infectious mononucleosis in a family. N Engl J Med 290:363\u2013367","journal-title":"N Engl J Med"},{"key":"316_CR17","doi-asserted-by":"crossref","first-page":"146","DOI":"10.3389\/fimmu.2019.00146","volume":"10","author":"MHM Barros","year":"2019","unstructured":"Barros MHM, Vera-Lozada G, Segges P, Hassan R, Niedobitek G (2019) Revisiting the tissue microenvironment of infectious mononucleosis: identification of EBV infection in T cells and deep characterization of immune profiles. Front Immunol 10:146","journal-title":"Front Immunol"},{"key":"316_CR18","doi-asserted-by":"crossref","first-page":"eabd4585","DOI":"10.1126\/science.abd4585","volume":"370","author":"P Bastard","year":"2020","unstructured":"Bastard P et al (2020) Autoantibodies against type I IFNs in patients with life-threatening COVID-19. Science 370:eabd4585","journal-title":"Science"},{"key":"316_CR19","doi-asserted-by":"crossref","first-page":"607","DOI":"10.1111\/his.12677","volume":"67","author":"K Bienemann","year":"2015","unstructured":"Bienemann K, Borkhardt A, Klapper W, Oschlies I (2015) High incidence of Epstein-Barr virus (EBV)-positive Hodgkin lymphoma and Hodgkin lymphoma-like B-cell lymphoproliferations with EBV latency profile 2 in children with interleukin-2-inducible T-cell kinase deficiency. Histopathology 67:607\u2013616","journal-title":"Histopathology"},{"key":"316_CR20","doi-asserted-by":"crossref","first-page":"e20220979","DOI":"10.1084\/jem.20220979","volume":"222","author":"E Blanco","year":"2025","unstructured":"Blanco E et al (2025) Dominant negative variants in ITPR3 impair T cell Ca2+ dynamics causing combined immunodeficiency. J Exp Med 222:e20220979","journal-title":"J Exp Med"},{"key":"316_CR21","doi-asserted-by":"crossref","first-page":"9865","DOI":"10.1073\/pnas.1817815116","volume":"116","author":"E Blommaert","year":"2019","unstructured":"Blommaert E et al (2019) Mutations in MAGT1 lead to a glycosylation disorder with a variable phenotype. Proc Natl Acad Sci USA 116:9865\u20139870","journal-title":"Proc Natl Acad Sci USA"},{"key":"316_CR22","doi-asserted-by":"crossref","first-page":"1279","DOI":"10.1007\/s00439-021-02400-1","volume":"141","author":"E Blommaert","year":"2022","unstructured":"Blommaert E et al (2022) Lack of NKG2D in MAGT1-deficient patients is caused by hypoglycosylation. Hum Genet 141:1279\u20131286","journal-title":"Hum Genet"},{"key":"316_CR259","doi-asserted-by":"publisher","unstructured":"Boisson-Dupuis Paul, St\u00e9phanie, Bastard Vivien, B\u00e9ziat Jacinta, Bustamante Aur\u00e9lie, Cobat Emmanuelle, Jouanguy Anne, Puel J\u00e9r\u00e9mie, Rosain Qian, Zhang Shen-Ying, Zhang Bertrand, Boisson (2025) The monogenic landscape of human infectious diseases. J Allergy Clin Immunol 155(3):768\u2013783. https:\/\/doi.org\/10.1016\/j.jaci.2024.12.1078","DOI":"10.1016\/j.jaci.2024.12.1078"},{"key":"316_CR23","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1084\/jem.194.3.235","volume":"194","author":"C Bottino","year":"2001","unstructured":"Bottino C et al (2001) NTB-A [correction of GNTB-A], a novel SH2D1A-associated surface molecule contributing to the inability of natural killer cells to kill Epstein-Barr virus-infected B cells in X-linked lymphoproliferative disease. J Exp Med 194:235\u2013246","journal-title":"J Exp Med"},{"key":"316_CR24","doi-asserted-by":"crossref","first-page":"533","DOI":"10.1007\/s10875-016-0306-1","volume":"36","author":"H Boztug","year":"2016","unstructured":"Boztug H et al (2016) NF-kappaB1 haploinsufficiency causing immunodeficiency and EBV-driven lymphoproliferation. J Clin Immunol 36:533\u2013540","journal-title":"J Clin Immunol"},{"key":"316_CR25","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1007\/s10875-016-0341-y","volume":"37","author":"I Brigida","year":"2017","unstructured":"Brigida I et al (2017) Large deletion of MAGT1 gene in a patient with classic Kaposi sarcoma, CD4 lymphopenia, and EBV infection. J Clin Immunol 37:32\u201335","journal-title":"J Clin Immunol"},{"key":"316_CR26","doi-asserted-by":"crossref","first-page":"543","DOI":"10.1309\/E343-KMYX-W3Y2-10KY","volume":"115","author":"K Broll","year":"2001","unstructured":"Broll K, Richter G, Pauly S, Hofstaedter F, Schwarz H (2001) CD137 expression in tumor vessel walls. High correlation with malignant tumors. Am J Clin Pathol 115:543\u2013549","journal-title":"Am J Clin Pathol"},{"key":"316_CR27","doi-asserted-by":"crossref","first-page":"680","DOI":"10.1007\/s10875-020-00940-1","volume":"41","author":"JP Brooks","year":"2021","unstructured":"Brooks JP et al (2021) Uncontrolled Epstein-Barr virus as an atypical presentation of deficiency in ADA2 (DADA2). J Clin Immunol 41:680\u2013683","journal-title":"J Clin Immunol"},{"key":"316_CR28","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1038\/bmt.2016.185","volume":"52","author":"D Cagdas","year":"2017","unstructured":"Cagdas D et al (2017) Course of IL-2-inducible T-cell kinase deficiency in a family: lymphomatoid granulomatosis, lymphoma and allogeneic bone marrow transplantation in one sibling; and death in the other. Bone Marrow Transplant 52:126\u2013129","journal-title":"Bone Marrow Transplant"},{"key":"316_CR29","doi-asserted-by":"crossref","first-page":"3752","DOI":"10.1016\/j.jaip.2021.05.032","volume":"9","author":"D Cagdas","year":"2021","unstructured":"Cagdas D et al (2021) Diversity in serine\/threonine protein kinase-4 deficiency and review of the literature. J Allergy Clin Immunol Pract 9:3752\u20133766 e3754","journal-title":"J Allergy Clin Immunol Pract"},{"key":"316_CR30","doi-asserted-by":"crossref","first-page":"1395","DOI":"10.1084\/jem.187.9.1395","volume":"187","author":"MF Callan","year":"1998","unstructured":"Callan MF et al (1998) Direct visualization of antigen-specific CD8+ T cells during the primary immune response to Epstein-Barr virus in vivo. J Exp Med 187:1395\u20131402","journal-title":"J Exp Med"},{"key":"316_CR31","doi-asserted-by":"crossref","first-page":"1251","DOI":"10.1172\/JCI10590","volume":"106","author":"MF Callan","year":"2000","unstructured":"Callan MF et al (2000) CD8(+) T-cell selection, function, and death in the primary immune response in vivo. J Clin Invest 106:1251\u20131261","journal-title":"J Clin Invest"},{"key":"316_CR32","doi-asserted-by":"crossref","first-page":"1475","DOI":"10.1016\/j.jpeds.2014.01.056","volume":"164","author":"C Cancrini","year":"2014","unstructured":"Cancrini C et al (2014) Clinical features and follow-up in patients with 22q11.2 deletion syndrome. J Pediatr 164:1475\u20131480 e1472","journal-title":"J Pediatr"},{"key":"316_CR33","doi-asserted-by":"crossref","first-page":"1332","DOI":"10.1182\/blood.2019000936","volume":"135","author":"SW Canna","year":"2020","unstructured":"Canna SW, Marsh RA (2020) Pediatric hemophagocytic lymphohistiocytosis. Blood 135:1332\u20131343","journal-title":"Blood"},{"key":"316_CR34","doi-asserted-by":"crossref","first-page":"1140","DOI":"10.1038\/ng.3089","volume":"46","author":"SW Canna","year":"2014","unstructured":"Canna SW et al (2014) An activating NLRC4 inflammasome mutation causes autoinflammation with recurrent macrophage activation syndrome. Nat Genet 46:1140\u20131146","journal-title":"Nat Genet"},{"key":"316_CR35","doi-asserted-by":"crossref","first-page":"2566","DOI":"10.1002\/cncr.22718","volume":"109","author":"S Cannella","year":"2007","unstructured":"Cannella S et al (2007) Germline mutations of the perforin gene are a frequent occurrence in childhood anaplastic large cell lymphoma. Cancer 109:2566\u20132571","journal-title":"Cancer"},{"key":"316_CR36","doi-asserted-by":"crossref","first-page":"665","DOI":"10.1146\/annurev-immunol-030409-101302","volume":"29","author":"JL Cannons","year":"2011","unstructured":"Cannons JL, Tangye SG, Schwartzberg PL (2011) SLAM family receptors and SAP adaptors in immunity. Annu Rev Immunol 29:665\u2013705","journal-title":"Annu Rev Immunol"},{"key":"316_CR37","doi-asserted-by":"crossref","first-page":"2015","DOI":"10.3389\/fimmu.2017.02015","volume":"8","author":"R Caorsi","year":"2017","unstructured":"Caorsi R et al (2017) CD70 deficiency due to a novel mutation in a patient with severe chronic EBV infection presenting as a periodic fever. Front Immunol 8:2015","journal-title":"Front Immunol"},{"key":"316_CR38","doi-asserted-by":"crossref","first-page":"186","DOI":"10.1126\/science.1240094","volume":"341","author":"B Chaigne-Delalande","year":"2013","unstructured":"Chaigne-Delalande B et al (2013) Mg2+ regulates cytotoxic functions of NK and CD8 T cells in chronic EBV infection through NKG2D. Science 341:186\u2013191","journal-title":"Science"},{"key":"316_CR39","doi-asserted-by":"crossref","first-page":"27350","DOI":"10.1074\/jbc.M111.221010","volume":"286","author":"P Charlot-Rabiega","year":"2011","unstructured":"Charlot-Rabiega P, Bardel E, Dietrich C, Kastelein R, Devergne O (2011) Signaling events involved in interleukin 27 (IL-27)-induced proliferation of human naive CD4+ T cells and B cells. J Biol Chem 286:27350\u201327362","journal-title":"J Biol Chem"},{"key":"316_CR40","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1097\/PAP.0000000000000284","volume":"28","author":"JH Choi","year":"2021","unstructured":"Choi JH, Ro JY (2021) The 2020 WHO classification of tumors of soft tissue: selected changes and new entities. Adv Anat Pathol 28:44\u201358","journal-title":"Adv Anat Pathol"},{"key":"316_CR41","doi-asserted-by":"crossref","first-page":"2600","DOI":"10.1182\/blood-2013-01-480665","volume":"122","author":"BK Chung","year":"2013","unstructured":"Chung BK et al (2013) Innate immune control of EBV-infected B cells by invariant natural killer T cells. Blood 122:2600\u20132608","journal-title":"Blood"},{"key":"316_CR42","doi-asserted-by":"crossref","first-page":"e359","DOI":"10.1097\/MPH.0000000000000073","volume":"36","author":"B Ciambotti","year":"2014","unstructured":"Ciambotti B et al (2014) Monoallelic mutations of the perforin gene may represent a predisposing factor to childhood anaplastic large cell lymphoma. J Pediatr Hematol Oncol 36:e359\u2013e365","journal-title":"J Pediatr Hematol Oncol"},{"key":"316_CR43","doi-asserted-by":"crossref","first-page":"4424","DOI":"10.1182\/blood-2004-04-1477","volume":"105","author":"R Clementi","year":"2005","unstructured":"Clementi R et al (2005) A proportion of patients with lymphoma may harbor mutations of the perforin gene. Blood 105:4424\u20134428","journal-title":"Blood"},{"key":"316_CR44","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1038\/2424","volume":"20","author":"AJ Coffey","year":"1998","unstructured":"Coffey AJ et al (1998) Host response to EBV infection in X-linked lymphoproliferative disease results from mutations in an SH2-domain encoding gene. Nat Genet 20:129\u2013135","journal-title":"Nat Genet"},{"key":"316_CR45","doi-asserted-by":"crossref","first-page":"1869","DOI":"10.3389\/fimmu.2017.01869","volume":"8","author":"JI Cohen","year":"2017","unstructured":"Cohen JI (2017) GATA2 deficiency and Epstein-Barr virus disease. Front Immunol 8:1869","journal-title":"Front Immunol"},{"key":"316_CR46","doi-asserted-by":"crossref","first-page":"5835","DOI":"10.1182\/blood-2010-11-316745","volume":"117","author":"JI Cohen","year":"2011","unstructured":"Cohen JI et al (2011) Characterization and treatment of chronic active Epstein-Barr virus disease: a 28-year experience in the United States. Blood 117:5835\u20135849","journal-title":"Blood"},{"key":"316_CR47","doi-asserted-by":"crossref","first-page":"445","DOI":"10.1007\/s10875-015-0168-y","volume":"35","author":"JI Cohen","year":"2015","unstructured":"Cohen JI et al (2015) Late-onset severe chronic active EBV in a patient for five years with mutations in STXBP2 (MUNC18-2) and PRF1 (perforin 1). J Clin Immunol 35:445\u2013448","journal-title":"J Clin Immunol"},{"key":"316_CR48","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1093\/cid\/ciw160","volume":"63","author":"JI Cohen","year":"2016","unstructured":"Cohen JI et al (2016) Association of GATA2 deficiency with severe primary Epstein-Barr virus (EBV) infection and EBV-associated cancers. Clin Infect Dis 63:41\u201347","journal-title":"Clin Infect Dis"},{"key":"316_CR49","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1182\/blood.2020005611","volume":"137","author":"PJ Collins","year":"2021","unstructured":"Collins PJ et al (2021) Characterizing EBV-associated lymphoproliferative diseases and the role of myeloid-derived suppressor cells. Blood 137:203\u2013215","journal-title":"Blood"},{"key":"316_CR50","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1126\/science.aay5663","volume":"369","author":"SA Cook","year":"2020","unstructured":"Cook SA et al (2020) HEM1 deficiency disrupts mTORC2 and F-actin control in inherited immunodysregulatory disease. Science 369:202\u2013207","journal-title":"Science"},{"key":"316_CR51","doi-asserted-by":"crossref","first-page":"1583","DOI":"10.1007\/s10875-022-01327-0","volume":"42","author":"S Cook","year":"2022","unstructured":"Cook S, Lenardo MJ, Freeman AF (2022) HEM1 actin immunodysregulatory disorder: genotypes, phenotypes, and future directions. J Clin Immunol 42:1583\u20131592","journal-title":"J Clin Immunol"},{"key":"316_CR52","doi-asserted-by":"crossref","first-page":"597","DOI":"10.1016\/j.jaci.2016.06.021","volume":"139","author":"TI Coulter","year":"2017","unstructured":"Coulter TI et al (2017) Clinical spectrum and features of activated phosphoinositide 3-kinase delta syndrome: a large patient cohort study. J Allergy Clin Immunol 139:597\u2013606 e594","journal-title":"J Allergy Clin Immunol"},{"key":"316_CR53","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0044010","volume":"7","author":"A Crequer","year":"2012","unstructured":"Crequer A et al (2012) Inherited MST1 deficiency underlies susceptibility to EV-HPV infections. PLoS One 7:e44010","journal-title":"PLoS One"},{"key":"316_CR54","doi-asserted-by":"crossref","first-page":"1278","DOI":"10.1002\/emmm.201303090","volume":"5","author":"RB Damgaard","year":"2013","unstructured":"Damgaard RB et al (2013) Disease-causing mutations in the XIAP BIR2 domain impair NOD2-dependent immune signalling. EMBO Mol Med 5:1278\u20131295","journal-title":"EMBO Mol Med"},{"key":"316_CR55","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1007\/s10875-016-0232-2","volume":"36","author":"TS Dang","year":"2016","unstructured":"Dang TS et al (2016) Defective leukocyte adhesion and chemotaxis contributes to combined immunodeficiency in humans with autosomal recessive MST1 deficiency. J Clin Immunol 36:117\u2013122","journal-title":"J Clin Immunol"},{"key":"316_CR56","doi-asserted-by":"crossref","first-page":"452","DOI":"10.1182\/bloodadvances.2021005291","volume":"6","author":"BJ Davila Saldana","year":"2022","unstructured":"Davila Saldana BJ et al (2022) High risk of relapsed disease in patients with NK\/T-cell chronic active Epstein-Barr virus disease outside of Asia. Blood Adv 6:452\u2013459","journal-title":"Blood Adv"},{"key":"316_CR57","doi-asserted-by":"crossref","first-page":"2544","DOI":"10.1182\/blood-2013-12-546309","volume":"124","author":"NF de Miranda","year":"2014","unstructured":"de Miranda NF et al (2014) Exome sequencing reveals novel mutation targets in diffuse large B-cell lymphomas derived from Chinese patients. Blood 124:2544\u20132553","journal-title":"Blood"},{"key":"316_CR58","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1002\/eji.201040905","volume":"41","author":"A DeBarros","year":"2011","unstructured":"DeBarros A, Chaves-Ferreira M, d\u2019Orey F, Ribot JC, Silva-Santos B (2011) CD70-CD27 interactions provide survival and proliferative signals that regulate T cell receptor-driven activation of human gammadelta peripheral blood lymphocytes. Eur J Immunol 41:195\u2013201","journal-title":"Eur J Immunol"},{"key":"316_CR59","doi-asserted-by":"crossref","first-page":"3225","DOI":"10.1182\/blood.2020009482","volume":"137","author":"Y Deng","year":"2021","unstructured":"Deng Y et al (2021) CD27 is required for protective lytic EBV antigen-specific CD8+ T-cell expansion. Blood 137:3225\u20133236","journal-title":"Blood"},{"key":"316_CR60","doi-asserted-by":"crossref","first-page":"1143","DOI":"10.1128\/jvi.70.2.1143-1153.1996","volume":"70","author":"O Devergne","year":"1996","unstructured":"Devergne O et al (1996) A novel interleukin-12 p40-related protein induced by latent Epstein-Barr virus infection in B lymphocytes. J Virol 70:1143\u20131153","journal-title":"J Virol"},{"key":"316_CR61","doi-asserted-by":"crossref","first-page":"12041","DOI":"10.1073\/pnas.94.22.12041","volume":"94","author":"O Devergne","year":"1997","unstructured":"Devergne O, Birkenbach M, Kieff E (1997) Epstein-Barr virus-induced gene 3 and the p35 subunit of interleukin 12 form a novel heterodimeric hematopoietin. Proc Natl Acad Sci USA 94:12041\u201312046","journal-title":"Proc Natl Acad Sci USA"},{"key":"316_CR62","doi-asserted-by":"crossref","first-page":"112","DOI":"10.1007\/s10875-014-0116-2","volume":"35","author":"F Dhalla","year":"2015","unstructured":"Dhalla F et al (2015) Identification of a novel mutation in MAGT1 and progressive multifocal leucoencephalopathy in a 58-year-old man with XMEN disease. J Clin Immunol 35:112\u2013118","journal-title":"J Clin Immunol"},{"key":"316_CR63","doi-asserted-by":"crossref","first-page":"1183","DOI":"10.1182\/blood.2023023707","volume":"144","author":"D Dissanayake","year":"2024","unstructured":"Dissanayake D et al (2024) Interleukin-1-mediated hyperinflammation in XIAP deficiency is associated with defective autophagy. Blood 144:1183\u20131192","journal-title":"Blood"},{"key":"316_CR64","doi-asserted-by":"crossref","first-page":"1827","DOI":"10.1084\/jem.20161017","volume":"214","author":"Z Djaoud","year":"2017","unstructured":"Djaoud Z et al (2017) Two alternate strategies for innate immunity to Epstein-Barr virus: one using NK cells and the other NK cells and gammadelta T cells. J Exp Med 214:1827\u20131841","journal-title":"J Exp Med"},{"key":"316_CR65","doi-asserted-by":"crossref","first-page":"974","DOI":"10.1016\/j.immuni.2012.03.023","volume":"36","author":"Z Dong","year":"2012","unstructured":"Dong Z et al (2012) The adaptor SAP controls NK cell activation by regulating the enzymes Vav-1 and SHIP-1 and by enhancing conjugates with target cells. Immunity 36:974\u2013985","journal-title":"Immunity"},{"key":"316_CR66","doi-asserted-by":"crossref","first-page":"64","DOI":"10.2350\/10-01-0787-CR.1","volume":"14","author":"S Du","year":"2011","unstructured":"Du S et al (2011) Hodgkin\u2019s and non-Hodgkin\u2019s lymphomas occurring in two brothers with Wiskott-Aldrich syndrome and review of the literature. Pediatr Dev Pathol 14:64\u201370","journal-title":"Pediatr Dev Pathol"},{"key":"316_CR67","first-page":"211","volume":"390","author":"SK Dunmire","year":"2015","unstructured":"Dunmire SK, Hogquist KA, Balfour HH (2015) Infectious mononucleosis. Curr Top Microbiol Immunol 390:211\u2013240","journal-title":"Curr Top Microbiol Immunol"},{"key":"316_CR68","doi-asserted-by":"crossref","first-page":"4383","DOI":"10.1182\/blood-2004-08-3269","volume":"105","author":"L Dupre","year":"2005","unstructured":"Dupre L et al (2005) SAP controls the cytolytic activity of CD8+ T cells against EBV-infected cells. Blood 105:4383\u20134389","journal-title":"Blood"},{"key":"316_CR69","doi-asserted-by":"crossref","first-page":"276","DOI":"10.1016\/j.jaci.2018.04.030","volume":"143","author":"ESJ Edwards","year":"2019","unstructured":"Edwards ESJ et al (2019) Activating PIK3CD mutations impair human cytotoxic lymphocyte differentiation and function and EBV immunity. J Allergy Clin Immunol 143:276\u2013291 e276","journal-title":"J Allergy Clin Immunol"},{"key":"316_CR70","doi-asserted-by":"crossref","first-page":"391","DOI":"10.1007\/s10875-019-00632-5","volume":"39","author":"A Eken","year":"2019","unstructured":"Eken A et al (2019) Genetic deficiency and biochemical inhibition of ITK affect human Th17, Treg, and innate lymphoid cells. J Clin Immunol 39:391\u2013400","journal-title":"J Clin Immunol"},{"key":"316_CR71","doi-asserted-by":"crossref","first-page":"1415","DOI":"10.3390\/v16091415","volume":"16","author":"A El Kettani","year":"2024","unstructured":"El Kettani A et al (2024) Case report of two independent Moroccan families with syndromic epidermodysplasia verruciformis and STK4 deficiency. Viruses 16:1415","journal-title":"Viruses"},{"key":"316_CR1000","doi-asserted-by":"crossref","unstructured":"Epstein MA, Achong BG, Barr YM (1964) Virus particles in cultured lymphoblasts from Burkitt\u2019s lymphoma. Lancet 1:702\u2013703","DOI":"10.1016\/S0140-6736(64)91524-7"},{"key":"316_CR72","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1007\/s10875-019-00598-4","volume":"39","author":"M Fang","year":"2019","unstructured":"Fang M et al (2019) Compound heterozygous mutations of IL2-inducible T cell kinase in a Swedish patient: the importance of early genetic diagnosis. J Clin Immunol 39:131\u2013134","journal-title":"J Clin Immunol"},{"key":"316_CR73","doi-asserted-by":"crossref","first-page":"1555","DOI":"10.1128\/JVI.73.2.1555-1564.1999","volume":"73","author":"GC Faulkner","year":"1999","unstructured":"Faulkner GC et al (1999) X-linked agammaglobulinemia patients are not infected with Epstein-Barr virus: implications for the biology of the virus. J Virol 73:1555\u20131564","journal-title":"J Virol"},{"key":"316_CR74","first-page":"6587323","volume":"2021","author":"M Forster","year":"2021","unstructured":"Forster M, Moran T, Beaven A, Voorhees T (2021) Novel ZAP-70-related immunodeficiency presenting with Epstein-Barr virus lymphoproliferative disorder and hemophagocytic lymphohistiocytosis. Case Rep Immunol 2021:6587323","journal-title":"Case Rep Immunol"},{"key":"316_CR75","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1016\/j.coi.2021.04.003","volume":"72","author":"B Fournier","year":"2021","unstructured":"Fournier B, Latour S (2021) Immunity to EBV as revealed by immunedeficiencies. Curr Opin Immunol 72:107\u2013115","journal-title":"Curr Opin Immunol"},{"key":"316_CR76","doi-asserted-by":"crossref","first-page":"e20192262","DOI":"10.1084\/jem.20192262","volume":"217","author":"B Fournier","year":"2020","unstructured":"Fournier B et al (2020) Rapid identification and characterization of infected cells in blood during chronic active Epstein-Barr virus infection. J Exp Med 217:e20192262","journal-title":"J Exp Med"},{"key":"316_CR77","doi-asserted-by":"crossref","first-page":"740","DOI":"10.1016\/j.jaci.2020.05.052","volume":"147","author":"B Fournier","year":"2021","unstructured":"Fournier B et al (2021) DEF6 deficiency, a mendelian susceptibility to EBV infection, lymphoma, and autoimmunity. J Allergy Clin Immunol 147:740\u2013743 e749","journal-title":"J Allergy Clin Immunol"},{"key":"316_CR78","doi-asserted-by":"crossref","first-page":"e20211682","DOI":"10.1084\/jem.20211682","volume":"219","author":"B Fournier","year":"2022","unstructured":"Fournier B et al (2022) Inherited TNFSF9 deficiency causes broad Epstein-Barr virus infection with EBV+ smooth muscle tumors. J Exp Med 219:e20211682","journal-title":"J Exp Med"},{"key":"316_CR79","doi-asserted-by":"crossref","first-page":"1523","DOI":"10.4049\/jimmunol.1102507","volume":"188","author":"S Fuchs","year":"2012","unstructured":"Fuchs S et al (2012) Antiviral and regulatory T cell immunity in a patient with stromal interaction molecule 1 deficiency. J Immunol 188:1523\u20131533","journal-title":"J Immunol"},{"key":"316_CR80","doi-asserted-by":"crossref","first-page":"118","DOI":"10.1007\/s10875-024-01712-x","volume":"44","author":"LS Gardner","year":"2024","unstructured":"Gardner LS et al (2024) Development of EBV related diffuse large B-cell lymphoma in deficiency of adenosine deaminase 2 with uncontrolled EBV infection. J Clin Immunol 44:118","journal-title":"J Clin Immunol"},{"key":"316_CR81","doi-asserted-by":"crossref","first-page":"614","DOI":"10.1182\/blood-2012-09-456095","volume":"121","author":"S Gerart","year":"2013","unstructured":"Gerart S et al (2013) Human iNKT and MAIT cells exhibit a PLZF-dependent proapoptotic propensity that is counterbalanced by XIAP. Blood 121:614\u2013623","journal-title":"Blood"},{"key":"316_CR82","doi-asserted-by":"crossref","first-page":"892","DOI":"10.1007\/s10875-014-0110-8","volume":"34","author":"S Ghosh","year":"2014","unstructured":"Ghosh S, Bienemann K, Boztug K, Borkhardt A (2014) Interleukin-2-inducible T-cell kinase (ITK) deficiency \u2013 clinical and molecular aspects. J Clin Immunol 34:892\u2013899","journal-title":"J Clin Immunol"},{"key":"316_CR83","doi-asserted-by":"crossref","first-page":"979","DOI":"10.3389\/fimmu.2018.00979","volume":"9","author":"S Ghosh","year":"2018","unstructured":"Ghosh S et al (2018) Interleukin-2-inducible T-cell kinase deficiency-new patients, new insight? Front Immunol 9:979","journal-title":"Front Immunol"},{"key":"316_CR84","doi-asserted-by":"crossref","first-page":"2638","DOI":"10.1182\/blood.2020006738","volume":"136","author":"S Ghosh","year":"2020","unstructured":"Ghosh S et al (2020) Extended clinical and immunological phenotype and transplant outcome in CD27 and CD70 deficiency. Blood 136:2638\u20132655","journal-title":"Blood"},{"key":"316_CR85","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1007\/s10875-024-01749-y","volume":"44","author":"K Golloshi","year":"2024","unstructured":"Golloshi K et al (2024) HLH and recurrent EBV lymphoma as the presenting manifestation of MAGT1 deficiency: a systematic review of the expanding disease spectrum. J Clin Immunol 44:153","journal-title":"J Clin Immunol"},{"key":"316_CR86","doi-asserted-by":"crossref","first-page":"1839","DOI":"10.1007\/s10875-021-01115-2","volume":"41","author":"A Guennoun","year":"2021","unstructured":"Guennoun A et al (2021) A novel STK4 mutation impairs T cell immunity through dysregulation of cytokine-induced adhesion and chemotaxis genes. J Clin Immunol 41:1839\u20131852","journal-title":"J Clin Immunol"},{"key":"316_CR87","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1002\/ijc.2910300610","volume":"30","author":"S Harada","year":"1982","unstructured":"Harada S, Bechtold T, Seeley JK, Purtilo DT (1982) Cell-mediated immunity to Epstein-Barr virus (EBV) and natural killer (NK)-cell activity in the X-linked lymphoproliferative syndrome. Int J Cancer 30:739\u2013744","journal-title":"Int J Cancer"},{"key":"316_CR88","doi-asserted-by":"crossref","first-page":"e02196","DOI":"10.1128\/JVI.02196-17","volume":"92","author":"JA Harker","year":"2018","unstructured":"Harker JA et al (2018) Interleukin-27R signaling mediates early viral containment and impacts innate and adaptive immunity after chronic lymphocytic choriomeningitis virus infection. J Virol 92:e02196","journal-title":"J Virol"},{"key":"316_CR89","doi-asserted-by":"crossref","first-page":"587","DOI":"10.1146\/annurev.immunol.25.022106.141553","volume":"25","author":"AD Hislop","year":"2007","unstructured":"Hislop AD, Taylor GS, Sauce D, Rickinson AB (2007) Cellular responses to viral infection in humans: lessons from Epstein-Barr virus. Annu Rev Immunol 25:587\u2013617","journal-title":"Annu Rev Immunol"},{"key":"316_CR90","doi-asserted-by":"crossref","first-page":"3249","DOI":"10.1182\/blood-2009-09-238832","volume":"116","author":"AD Hislop","year":"2010","unstructured":"Hislop AD et al (2010) Impaired Epstein-Barr virus-specific CD8+ T-cell function in X-linked lymphoproliferative disease is restricted to SLAM family-positive B-cell targets. Blood 116:3249\u20133257","journal-title":"Blood"},{"key":"316_CR91","doi-asserted-by":"crossref","first-page":"244","DOI":"10.1016\/j.jcv.2010.11.017","volume":"50","author":"Y Hoshino","year":"2011","unstructured":"Hoshino Y, Nishikawa K, Ito Y, Kuzushima K, Kimura H (2011) Kinetics of Epstein-Barr virus load and virus-specific CD8+ T cells in acute infectious mononucleosis. J Clin Virol 50:244\u2013246","journal-title":"J Clin Virol"},{"key":"316_CR92","doi-asserted-by":"crossref","first-page":"825","DOI":"10.1093\/infdis\/jiy231","volume":"218","author":"A Hoshino","year":"2018","unstructured":"Hoshino A et al (2018) Dysregulation of Epstein-Barr virus infection in hypomorphic ZAP70 mutation. J Infect Dis 218:825\u2013834","journal-title":"J Infect Dis"},{"key":"316_CR93","doi-asserted-by":"crossref","first-page":"421","DOI":"10.1016\/j.jaci.2018.07.044","volume":"143","author":"A Hoshino","year":"2019","unstructured":"Hoshino A et al (2019) Modification of cellular and humoral immunity by somatically reverted T cells in X-linked lymphoproliferative syndrome type 1. J Allergy Clin Immunol 143:421\u2013424 e411","journal-title":"J Allergy Clin Immunol"},{"key":"316_CR94","doi-asserted-by":"crossref","first-page":"2000","DOI":"10.3389\/fimmu.2019.02000","volume":"10","author":"MK Howe","year":"2019","unstructured":"Howe MK et al (2019) Magnesium restores activity to peripheral blood cells in a patient with functionally impaired interleukin-2-inducible T cell kinase. Front Immunol 10:2000","journal-title":"Front Immunol"},{"key":"316_CR95","doi-asserted-by":"crossref","first-page":"1791","DOI":"10.1084\/jem.20190173","volume":"216","author":"Z Huang","year":"2019","unstructured":"Huang Z et al (2019) IL-27 promotes the expansion of self-renewing CD8(+) T cells in persistent viral infection. J Exp Med 216:1791\u20131808","journal-title":"J Exp Med"},{"key":"316_CR96","doi-asserted-by":"crossref","first-page":"1350","DOI":"10.1172\/JCI37901","volume":"119","author":"K Huck","year":"2009","unstructured":"Huck K et al (2009) Girls homozygous for an IL-2-inducible T cell kinase mutation that leads to protein deficiency develop fatal EBV-associated lymphoproliferation. J Clin Invest 119:1350\u20131358","journal-title":"J Clin Invest"},{"key":"316_CR97","doi-asserted-by":"crossref","first-page":"519","DOI":"10.1038\/modpathol.3800369","volume":"18","author":"SD Hudnall","year":"2005","unstructured":"Hudnall SD et al (2005) Distribution and phenotype of Epstein-Barr virus-infected cells in human pharyngeal tonsils. Mod Pathol 18:519\u2013527","journal-title":"Mod Pathol"},{"key":"316_CR98","doi-asserted-by":"crossref","first-page":"2417","DOI":"10.1016\/j.ejca.2014.06.006","volume":"50","author":"K Hussein","year":"2014","unstructured":"Hussein K, Rath B, Ludewig B, Kreipe H, Jonigk D (2014) Clinico-pathological characteristics of different types of immunodeficiency-associated smooth muscle tumours. Eur J Cancer 50:2417\u20132424","journal-title":"Eur J Cancer"},{"key":"316_CR99","doi-asserted-by":"crossref","first-page":"2037","DOI":"10.1016\/j.humpath.2010.03.014","volume":"42","author":"S Itoh","year":"2011","unstructured":"Itoh S, Ohno T, Kakizaki S, Ichinohasama R (2011) Epstein-Barr virus-positive T-cell lymphoma cells having chromosome 22q11.2 deletion: an autopsy report of DiGeorge syndrome. Hum Pathol 42:2037\u20132041","journal-title":"Hum Pathol"},{"key":"316_CR100","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1099\/vir.0.013482-0","volume":"91","author":"S Iwata","year":"2010","unstructured":"Iwata S et al (2010) Quantitative analysis of Epstein-Barr virus (EBV)-related gene expression in patients with chronic active EBV infection. J Gen Virol 91:42\u201350","journal-title":"J Gen Virol"},{"key":"316_CR101","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1084\/jem.20160784","volume":"214","author":"K Izawa","year":"2017","unstructured":"Izawa K et al (2017) Inherited CD70 deficiency in humans reveals a critical role for the CD70-CD27 pathway in immunity to Epstein-Barr virus infection. J Exp Med 214:73\u201389","journal-title":"J Exp Med"},{"key":"316_CR102","doi-asserted-by":"crossref","DOI":"10.1371\/journal.ppat.1004746","volume":"11","author":"S Jayasooriya","year":"2015","unstructured":"Jayasooriya S et al (2015) Early virological and immunological events in asymptomatic Epstein-Barr virus infection in African children. PLoS Pathog 11:e1004746","journal-title":"PLoS Pathog"},{"key":"316_CR103","doi-asserted-by":"crossref","first-page":"1061","DOI":"10.1038\/ng.3358","volume":"47","author":"L Jiang","year":"2015","unstructured":"Jiang L et al (2015) Exome sequencing identifies somatic mutations of DDX3X in natural killer\/T-cell lymphoma. Nat Genet 47:1061\u20131066","journal-title":"Nat Genet"},{"key":"316_CR104","doi-asserted-by":"crossref","first-page":"1908","DOI":"10.1111\/j.1600-6143.2012.04011.x","volume":"12","author":"D Jonigk","year":"2012","unstructured":"Jonigk D et al (2012) Molecular and clinicopathological analysis of Epstein-Barr virus-associated posttransplant smooth muscle tumors. Am J Transplant 12:1908\u20131917","journal-title":"Am J Transplant"},{"key":"316_CR105","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1111\/petr.12426","volume":"19","author":"J Jossen","year":"2015","unstructured":"Jossen J et al (2015) Epstein-Barr virus-associated smooth muscle tumors in children following solid organ transplantation: a review. Pediatr Transplant 19:235\u2013243","journal-title":"Pediatr Transplant"},{"key":"316_CR106","doi-asserted-by":"crossref","first-page":"927","DOI":"10.1007\/s10875-018-0559-y","volume":"38","author":"C Kamae","year":"2018","unstructured":"Kamae C et al (2018) Clinical and immunological characterization of ICF syndrome in Japan. J Clin Immunol 38:927\u2013937","journal-title":"J Clin Immunol"},{"key":"316_CR107","doi-asserted-by":"crossref","first-page":"1828","DOI":"10.1084\/jem.20181970","volume":"216","author":"C Kanellopoulou","year":"2019","unstructured":"Kanellopoulou C et al (2019) Mg(2+) regulation of kinase signaling and immune function. J Exp Med 216:1828\u20131842","journal-title":"J Exp Med"},{"key":"316_CR108","doi-asserted-by":"crossref","first-page":"2699","DOI":"10.4049\/jimmunol.1601202","volume":"198","author":"SM Kapnick","year":"2017","unstructured":"Kapnick SM, Stinchcombe JC, Griffiths GM, Schwartzberg PL (2017) Inducible T cell kinase regulates the acquisition of cytolytic capacity and degranulation in CD8(+) CTLs. J Immunol 198:2699\u20132711","journal-title":"J Immunol"},{"key":"316_CR109","doi-asserted-by":"crossref","first-page":"1244","DOI":"10.1182\/blood-2003-06-2171","volume":"103","author":"H Katano","year":"2004","unstructured":"Katano H et al (2004) Chronic active Epstein-Barr virus infection associated with mutations in perforin that impair its maturation. Blood 103:1244\u20131252","journal-title":"Blood"},{"key":"316_CR110","doi-asserted-by":"crossref","first-page":"3268","DOI":"10.1016\/j.jtha.2023.05.007","volume":"21","author":"A Kauskot","year":"2023","unstructured":"Kauskot A et al (2023) MAGT1 deficiency in XMEN disease is associated with severe platelet dysfunction and impaired platelet glycoprotein N-glycosylation. J Thromb Haemost 21:3268\u20133278","journal-title":"J Thromb Haemost"},{"key":"316_CR111","doi-asserted-by":"crossref","first-page":"599","DOI":"10.1016\/j.jaci.2020.12.656","volume":"148","author":"M Kaustio","year":"2021","unstructured":"Kaustio M et al (2021) Loss of DIAPH1 causes SCBMS, combined immunodeficiency, and mitochondrial dysfunction. J Allergy Clin Immunol 148:599\u2013611","journal-title":"J Allergy Clin Immunol"},{"key":"316_CR112","doi-asserted-by":"crossref","first-page":"1481","DOI":"10.1111\/j.1349-7006.2012.02305.x","volume":"103","author":"S Kawabe","year":"2012","unstructured":"Kawabe S et al (2012) Application of flow cytometric in situ hybridization assay to Epstein-Barr virus-associated T\/natural killer cell lymphoproliferative diseases. Cancer Sci 103:1481\u20131488","journal-title":"Cancer Sci"},{"key":"316_CR113","doi-asserted-by":"crossref","first-page":"568","DOI":"10.1007\/s12185-023-03660-5","volume":"118","author":"JI Kawada","year":"2023","unstructured":"Kawada JI et al (2023) Updated guidelines for chronic active Epstein-Barr virus disease. Int J Hematol 118:568\u2013576","journal-title":"Int J Hematol"},{"key":"316_CR114","volume":"3","author":"A Khoreva","year":"2024","unstructured":"Khoreva A et al (2024) Novel hemizygous CORO1A variant leads to combined immunodeficiency with defective platelet calcium signaling and cell mobility. J Allergy Clin Immunol Glob 3:100172","journal-title":"J Allergy Clin Immunol Glob"},{"key":"316_CR115","doi-asserted-by":"crossref","first-page":"71","DOI":"10.3389\/fped.2019.00071","volume":"7","author":"WY Kim","year":"2019","unstructured":"Kim WY, Montes-Mojarro IA, Fend F, Quintanilla-Martinez L (2019) Epstein-Barr virus-associated T and NK-cell lymphoproliferative diseases. Front Pediatr 7:71","journal-title":"Front Pediatr"},{"key":"316_CR116","doi-asserted-by":"crossref","first-page":"673","DOI":"10.1182\/blood-2011-10-381921","volume":"119","author":"H Kimura","year":"2012","unstructured":"Kimura H et al (2012) EBV-associated T\/NK-cell lymphoproliferative diseases in nonimmunocompromised hosts: prospective analysis of 108 cases. Blood 119:673\u2013686","journal-title":"Blood"},{"key":"316_CR117","doi-asserted-by":"crossref","first-page":"700","DOI":"10.1038\/s41419-019-1938-x","volume":"10","author":"J Knop","year":"2019","unstructured":"Knop J et al (2019) TNFR2 induced priming of the inflammasome leads to a RIPK1-dependent cell death in the absence of XIAP. Cell Death Dis 10:700","journal-title":"Cell Death Dis"},{"key":"316_CR118","doi-asserted-by":"crossref","first-page":"5422","DOI":"10.4049\/jimmunol.153.12.5422","volume":"153","author":"T Kobata","year":"1994","unstructured":"Kobata T, Agematsu K, Kameoka J, Schlossman SF, Morimoto C (1994) CD27 is a signal-transducing molecule involved in CD45RA+ naive T cell costimulation. J Immunol 153:5422\u20135432","journal-title":"J Immunol"},{"key":"316_CR119","doi-asserted-by":"crossref","first-page":"553","DOI":"10.3389\/fimmu.2020.00553","volume":"11","author":"I Kourtzelis","year":"2020","unstructured":"Kourtzelis I, Hajishengallis G, Chavakis T (2020) Phagocytosis of apoptotic cells in resolution of inflammation. Front Immunol 11:553","journal-title":"Front Immunol"},{"key":"316_CR120","doi-asserted-by":"crossref","first-page":"1593","DOI":"10.3389\/fimmu.2020.01593","volume":"11","author":"R Kruger","year":"2020","unstructured":"Kruger R et al (2020) CD70 deficiency associated with chronic Epstein-Barr virus infection, recurrent airway infections and severe gingivitis in a 24-year-old woman. Front Immunol 11:1593","journal-title":"Front Immunol"},{"key":"316_CR121","doi-asserted-by":"crossref","first-page":"575","DOI":"10.1158\/2326-6066.CIR-15-0098","volume":"3","author":"LL Lanier","year":"2015","unstructured":"Lanier LL (2015) NKG2D receptor and its ligands in host defense. Cancer Immunol Res 3:575\u2013582","journal-title":"Cancer Immunol Res"},{"key":"316_CR122","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1016\/j.semcdb.2015.01.015","volume":"39","author":"S Latour","year":"2015","unstructured":"Latour S, Aguilar C (2015) XIAP deficiency syndrome in humans. Semin Cell Dev Biol 39:115\u2013123","journal-title":"Semin Cell Dev Biol"},{"key":"316_CR123","doi-asserted-by":"crossref","first-page":"174","DOI":"10.1111\/imr.12791","volume":"291","author":"S Latour","year":"2019","unstructured":"Latour S, Fischer A (2019) Signaling pathways involved in the T-cell-mediated immunity against Epstein-Barr virus: lessons from genetic diseases. Immunol Rev 291:174\u2013189","journal-title":"Immunol Rev"},{"key":"316_CR124","doi-asserted-by":"crossref","first-page":"1103","DOI":"10.3389\/fimmu.2018.01103","volume":"9","author":"S Latour","year":"2018","unstructured":"Latour S, Winter S (2018) Inherited Immunodeficiencies with high predisposition to Epstein-Barr virus-driven lymphoproliferative diseases. Front Immunol 9:1103","journal-title":"Front Immunol"},{"key":"316_CR125","doi-asserted-by":"crossref","first-page":"681","DOI":"10.1038\/90615","volume":"2","author":"S Latour","year":"2001","unstructured":"Latour S et al (2001) Regulation of SLAM-mediated signal transduction by SAP, the X-linked lymphoproliferative gene product. Nat Immunol 2:681\u2013690","journal-title":"Nat Immunol"},{"key":"316_CR126","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1038\/ncb919","volume":"5","author":"S Latour","year":"2003","unstructured":"Latour S et al (2003) Binding of SAP SH2 domain to FynT SH3 domain reveals a novel mechanism of receptor signalling in immune regulation. Nat Cell Biol 5:149\u2013154","journal-title":"Nat Cell Biol"},{"key":"316_CR127","doi-asserted-by":"crossref","first-page":"948","DOI":"10.1007\/s10875-020-00812-8","volume":"40","author":"T Le Voyer","year":"2020","unstructured":"Le Voyer T et al (2020) Late-onset EBV susceptibility and refractory pure red cell aplasia revealing DADA2. J Clin Immunol 40:948\u2013953","journal-title":"J Clin Immunol"},{"key":"316_CR128","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1056\/NEJM199501053320104","volume":"332","author":"ES Lee","year":"1995","unstructured":"Lee ES et al (1995) The association of Epstein-Barr virus with smooth-muscle tumors occurring after organ transplantation. N Engl J Med 332:19\u201325","journal-title":"N Engl J Med"},{"key":"316_CR129","doi-asserted-by":"crossref","first-page":"229","DOI":"10.14740\/jh1284","volume":"13","author":"L Lerner","year":"2024","unstructured":"Lerner L et al (2024) Systemic Epstein-Barr virus-positive T-cell lymphoma of childhood associated with t(1;22)(p22;q11.2) mutation. J Hematol 13:229\u2013237","journal-title":"J Hematol"},{"key":"316_CR130","doi-asserted-by":"crossref","first-page":"625","DOI":"10.1007\/s10875-022-01412-4","volume":"43","author":"A Lev","year":"2023","unstructured":"Lev A et al (2023) SLP76 mutation associated with combined immunodeficiency and EBV-related lymphoma. J Clin Immunol 43:625\u2013635","journal-title":"J Clin Immunol"},{"key":"316_CR131","doi-asserted-by":"crossref","first-page":"e20220275","DOI":"10.1084\/jem.20220275","volume":"220","author":"R Levy","year":"2023","unstructured":"Levy R et al (2023) Human CARMIL2 deficiency underlies a broader immunological and clinical phenotype than CD28 deficiency. J Exp Med 220:e20220275","journal-title":"J Exp Med"},{"key":"316_CR132","doi-asserted-by":"crossref","first-page":"471","DOI":"10.1038\/nature10246","volume":"475","author":"FY Li","year":"2011","unstructured":"Li FY et al (2011) Second messenger role for Mg2+ revealed by human T-cell immunodeficiency. Nature 475:471\u2013476","journal-title":"Nature"},{"key":"316_CR133","doi-asserted-by":"crossref","first-page":"e121322","DOI":"10.1172\/jci.insight.121322","volume":"3","author":"G Li","year":"2018","unstructured":"Li G et al (2018) 4-1BB enhancement of CAR T function requires NF-kappaB and TRAFs. JCI Insight 3:e121322","journal-title":"JCI Insight"},{"key":"316_CR134","doi-asserted-by":"crossref","first-page":"963","DOI":"10.1038\/leu.2011.371","volume":"26","author":"RM Linka","year":"2012","unstructured":"Linka RM et al (2012) Loss-of-function mutations within the IL-2 inducible kinase ITK in patients with EBV-associated lymphoproliferative diseases. Leukemia 26:963\u2013971","journal-title":"Leukemia"},{"key":"316_CR135","volume":"112","author":"M Liu","year":"2023","unstructured":"Liu M, Wang R, Xie Z (2023) T cell-mediated immunity during Epstein-Barr virus infections in children. Infect Genet Evol 112:105443","journal-title":"Infect Genet Evol"},{"issue":"4","key":"316_CR136","doi-asserted-by":"crossref","first-page":"1346","DOI":"10.1016\/j.jaci.2024.11.029","volume":"155","author":"M Liu","year":"2025","unstructured":"Liu M et al (2025) Features of hyperinflammation link the biology of EBV infection and cytokine storm syndromes. J Allergy Clin Immunol 155(4):1346\u20131356.e9","journal-title":"J Allergy Clin Immunol"},{"key":"316_CR137","doi-asserted-by":"crossref","first-page":"447","DOI":"10.1038\/s41590-019-0334-0","volume":"20","author":"Y Lu","year":"2019","unstructured":"Lu Y et al (2019) SLAM receptors foster iNKT cell development by reducing TCR signal strength after positive selection. Nat Immunol 20:447\u2013457","journal-title":"Nat Immunol"},{"key":"316_CR138","doi-asserted-by":"crossref","first-page":"1657","DOI":"10.1002\/pbc.26048","volume":"63","author":"SH Lum","year":"2016","unstructured":"Lum SH et al (2016) Successful curative therapy with rituximab and allogeneic haematopoietic stem cell transplantation for MALT lymphoma associated with STK4-mutated CD4+ lymphocytopenia. Pediatr Blood Cancer 63:1657\u20131659","journal-title":"Pediatr Blood Cancer"},{"key":"316_CR139","doi-asserted-by":"crossref","first-page":"1096","DOI":"10.1016\/j.jaci.2022.11.012","volume":"151","author":"H Luo","year":"2023","unstructured":"Luo H et al (2023) Clinical and genetic characterization of Epstein-Barr virus-associated T\/NK-cell lymphoproliferative diseases. J Allergy Clin Immunol 151:1096\u20131109","journal-title":"J Allergy Clin Immunol"},{"key":"316_CR140","doi-asserted-by":"crossref","first-page":"984","DOI":"10.1016\/j.jaci.2023.06.015","volume":"152","author":"ME Maccari","year":"2023","unstructured":"Maccari ME et al (2023) Activated phosphoinositide 3-kinase delta syndrome: update from the ESID registry and comparison with other autoimmune-lymphoproliferative inborn errors of immunity. J Allergy Clin Immunol 152:984\u2013996 e910","journal-title":"J Allergy Clin Immunol"},{"key":"316_CR141","doi-asserted-by":"crossref","first-page":"6708","DOI":"10.1073\/pnas.1314975111","volume":"111","author":"EM Mace","year":"2014","unstructured":"Mace EM, Orange JS (2014) Lytic immune synapse function requires filamentous actin deconstruction by Coronin 1A. Proc Natl Acad Sci USA 111:6708\u20136713","journal-title":"Proc Natl Acad Sci USA"},{"key":"316_CR142","doi-asserted-by":"crossref","first-page":"368","DOI":"10.3389\/fimmu.2018.00368","volume":"9","author":"T Magg","year":"2018","unstructured":"Magg T et al (2018) Epstein-Barr virus(+) smooth muscle tumors as manifestation of primary immunodeficiency disorders. Front Immunol 9:368","journal-title":"Front Immunol"},{"key":"316_CR143","doi-asserted-by":"crossref","first-page":"418","DOI":"10.1159\/000333472","volume":"158","author":"D Mansouri","year":"2012","unstructured":"Mansouri D et al (2012) IL-2-inducible T-cell kinase deficiency with pulmonary manifestations due to disseminated Epstein-Barr virus infection. Int Arch Allergy Immunol 158:418\u2013422","journal-title":"Int Arch Allergy Immunol"},{"issue":"2","key":"316_CR144","doi-asserted-by":"crossref","first-page":"595","DOI":"10.1016\/j.jaci.2017.10.026","volume":"142","author":"H Mao","year":"2018","unstructured":"Mao H et al (2018) RASGRP1 mutation in autoimmune lymphoproliferative syndrome-like disease. J Allergy Clin Immunol 142(2):595\u2013604.e16","journal-title":"J Allergy Clin Immunol"},{"key":"316_CR145","doi-asserted-by":"crossref","first-page":"288","DOI":"10.1038\/nature13386","volume":"510","author":"E Martin","year":"2014","unstructured":"Martin E et al (2014) CTP synthase 1 deficiency in humans reveals its central role in lymphocyte proliferation. Nature 510:288\u2013292","journal-title":"Nature"},{"key":"316_CR146","doi-asserted-by":"crossref","first-page":"e133880","DOI":"10.1172\/jci.insight.133880","volume":"5","author":"E Martin","year":"2020","unstructured":"Martin E et al (2020) Impaired lymphocyte function and differentiation in CTPS1-deficient patients result from a hypomorphic homozygous mutation. JCI Insight 5:e133880","journal-title":"JCI Insight"},{"key":"316_CR147","doi-asserted-by":"crossref","first-page":"620","DOI":"10.1038\/s41586-024-07213-6","volume":"628","author":"E Martin","year":"2024","unstructured":"Martin E et al (2024) Role of IL-27 in Epstein-Barr virus infection revealed by IL-27RA deficiency. Nature 628:620\u2013629","journal-title":"Nature"},{"key":"316_CR148","doi-asserted-by":"crossref","first-page":"13638","DOI":"10.1074\/jbc.RA119.008903","volume":"294","author":"M Matsuda-Lennikov","year":"2019","unstructured":"Matsuda-Lennikov M et al (2019) Magnesium transporter 1 (MAGT1) deficiency causes selective defects in N-linked glycosylation and expression of immune-response genes. J Biol Chem 294:13638\u201313656","journal-title":"J Biol Chem"},{"key":"316_CR149","doi-asserted-by":"crossref","first-page":"12","DOI":"10.1056\/NEJM199501053320103","volume":"332","author":"KL McClain","year":"1995","unstructured":"McClain KL et al (1995) Association of Epstein-Barr virus with leiomyosarcomas in young people with AIDS. N Engl J Med 332:12\u201318","journal-title":"N Engl J Med"},{"key":"316_CR150","doi-asserted-by":"crossref","first-page":"1091","DOI":"10.1084\/jem.20171068","volume":"215","author":"AV Menk","year":"2018","unstructured":"Menk AV et al (2018) 4-1BB costimulation induces T cell mitochondrial function and biogenesis enabling cancer immunotherapeutic responses. J Exp Med 215:1091\u20131100","journal-title":"J Exp Med"},{"key":"316_CR151","doi-asserted-by":"crossref","first-page":"2280","DOI":"10.1182\/blood-2009-03-208215","volume":"114","author":"S Middendorp","year":"2009","unstructured":"Middendorp S et al (2009) Mice deficient for CD137 ligand are predisposed to develop germinal center-derived B-cell lymphoma. Blood 114:2280\u20132289","journal-title":"Blood"},{"key":"316_CR152","doi-asserted-by":"crossref","first-page":"e202302066","DOI":"10.26508\/lsa.202302066","volume":"6","author":"N Minet","year":"2023","unstructured":"Minet N et al (2023) Differential roles of CTP synthetases CTPS1 and CTPS2 in cell proliferation. Life Sci Alliance 6:e202302066","journal-title":"Life Sci Alliance"},{"key":"316_CR153","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1016\/j.exphem.2019.09.024","volume":"78","author":"W Mo","year":"2019","unstructured":"Mo W et al (2019) Application of blood and immunodeficiency gene detection in the diagnosis of hemophagocytic lymphohistiocytosis patients. Exp Hematol 78:62\u201369","journal-title":"Exp Hematol"},{"key":"316_CR154","first-page":"11","volume-title":"RASGRP1 deficiency associated with diffuse mesangial sclerosis infantile nephrotic syndrome and Epstein-Barr virus-induced Hodgkin\u2019s lymphoma","author":"KN Mohamed Nashrudin","year":"2025","unstructured":"Mohamed Nashrudin KN et al (2025) RASGRP1 deficiency associated with diffuse mesangial sclerosis infantile nephrotic syndrome and Epstein-Barr virus-induced Hodgkin\u2019s lymphoma, vol 38. Pediatr Allergy Immunol Pulmonol, p 11"},{"key":"316_CR155","doi-asserted-by":"crossref","first-page":"1594","DOI":"10.1016\/j.jaci.2013.01.042","volume":"131","author":"D Moshous","year":"2013","unstructured":"Moshous D et al (2013) Whole-exome sequencing identifies coronin-1A deficiency in 3 siblings with immunodeficiency and EBV-associated B-cell lymphoproliferation. J Allergy Clin Immunol 131:1594\u20131603","journal-title":"J Allergy Clin Immunol"},{"key":"316_CR156","volume":"9","author":"ACA Mudde","year":"2021","unstructured":"Mudde ACA, Booth C, Marsh RA (2021) Evolution of our understanding of XIAP deficiency. Front Pediatr 9:660520","journal-title":"Front Pediatr"},{"key":"316_CR157","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1016\/j.coviro.2017.07.026","volume":"25","author":"C Munz","year":"2017","unstructured":"Munz C (2017a) Humanized mouse models for Epstein Barr virus infection. Curr Opin Virol 25:113\u2013118","journal-title":"Curr Opin Virol"},{"key":"316_CR158","doi-asserted-by":"crossref","first-page":"1658","DOI":"10.3389\/fimmu.2017.01658","volume":"8","author":"C Munz","year":"2017","unstructured":"Munz C (2017b) Epstein-Barr virus-specific immune control by innate lymphocytes. Front Immunol 8:1658","journal-title":"Front Immunol"},{"key":"316_CR159","doi-asserted-by":"crossref","first-page":"691","DOI":"10.1038\/s41579-019-0249-7","volume":"17","author":"C Munz","year":"2019","unstructured":"Munz C (2019) Latency and lytic replication in Epstein-Barr virus-associated oncogenesis. Nat Rev Microbiol 17:691\u2013700","journal-title":"Nat Rev Microbiol"},{"key":"316_CR160","doi-asserted-by":"crossref","DOI":"10.1002\/rmv.2095","volume":"30","author":"T Murata","year":"2020","unstructured":"Murata T, Okuno Y, Sato Y, Watanabe T, Kimura H (2020) Oncogenesis of CAEBV revealed: intragenic deletions in the viral genome and leaky expression of lytic genes. Rev Med Virol 30:e2095","journal-title":"Rev Med Virol"},{"key":"316_CR161","doi-asserted-by":"crossref","first-page":"3458","DOI":"10.1182\/blood-2011-09-378364","volume":"119","author":"NT Nehme","year":"2012","unstructured":"Nehme NT et al (2012) MST1 mutations in autosomal recessive primary immunodeficiency characterized by defective naive T-cell survival. Blood 119:3458\u20133468","journal-title":"Blood"},{"key":"316_CR162","doi-asserted-by":"crossref","first-page":"13765","DOI":"10.1073\/pnas.95.23.13765","volume":"95","author":"KE Nichols","year":"1998","unstructured":"Nichols KE et al (1998) Inactivating mutations in an SH2 domain-encoding gene in X-linked lymphoproliferative syndrome. Proc Natl Acad Sci USA 95:13765\u201313770","journal-title":"Proc Natl Acad Sci USA"},{"key":"316_CR163","doi-asserted-by":"crossref","first-page":"340","DOI":"10.1038\/nm1189","volume":"11","author":"KE Nichols","year":"2005","unstructured":"Nichols KE et al (2005) Regulation of NKT cell development by SAP, the protein defective in XLP. Nat Med 11:340\u2013345","journal-title":"Nat Med"},{"key":"316_CR164","doi-asserted-by":"crossref","first-page":"455","DOI":"10.1038\/jhg.2013.56","volume":"58","author":"H Nitta","year":"2013","unstructured":"Nitta H et al (2013) Three novel ZBTB24 mutations identified in Japanese and Cape Verdean type 2 ICF syndrome patients. J Hum Genet 58:455\u2013460","journal-title":"J Hum Genet"},{"key":"316_CR165","doi-asserted-by":"crossref","first-page":"e20220484","DOI":"10.1084\/jem.20220484","volume":"220","author":"M Ogishi","year":"2023","unstructured":"Ogishi M et al (2023) Inherited human ITK deficiency impairs IFN-gamma immunity and underlies tuberculosis. J Exp Med 220:e20220484","journal-title":"J Exp Med"},{"key":"316_CR166","doi-asserted-by":"crossref","first-page":"404","DOI":"10.1038\/s41564-018-0334-0","volume":"4","author":"Y Okuno","year":"2019","unstructured":"Okuno Y et al (2019) Defective Epstein-Barr virus in chronic active infection and haematological malignancy. Nat Microbiol 4:404\u2013413","journal-title":"Nat Microbiol"},{"key":"316_CR167","doi-asserted-by":"crossref","first-page":"5350","DOI":"10.1158\/1078-0432.CCR-08-2979","volume":"15","author":"KW Ong","year":"2009","unstructured":"Ong KW et al (2009) Expression of EBV latent antigens, mammalian target of rapamycin, and tumor suppression genes in EBV-positive smooth muscle tumors: clinical and therapeutic implications. Clin Cancer Res 15:5350\u20135358","journal-title":"Clin Cancer Res"},{"key":"316_CR168","doi-asserted-by":"crossref","first-page":"1522","DOI":"10.1182\/blood-2010-07-298372","volume":"117","author":"J Pachlopnik Schmid","year":"2011","unstructured":"Pachlopnik Schmid J et al (2011) Clinical similarities and differences of patients with X-linked lymphoproliferative syndrome type 1 (XLP-1\/SAP deficiency) versus type 2 (XLP-2\/XIAP deficiency). Blood 117:1522\u20131529","journal-title":"Blood"},{"key":"316_CR169","doi-asserted-by":"crossref","DOI":"10.3389\/fimmu.2021.654167","volume":"12","author":"GL Padeira","year":"2021","unstructured":"Padeira GL et al (2021) Case report: primary immunodeficiencies, massive EBV+ T-cell lympoproliferation leading to the diagnosis of ICF2 syndrome. Front Immunol 12:654167","journal-title":"Front Immunol"},{"key":"316_CR170","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pbio.1001187","volume":"9","author":"U Palendira","year":"2011","unstructured":"Palendira U et al (2011) Molecular pathogenesis of EBV susceptibility in XLP as revealed by analysis of female carriers with heterozygous expression of SAP. PLoS Biol 9:e1001187","journal-title":"PLoS Biol"},{"key":"316_CR171","doi-asserted-by":"crossref","first-page":"913","DOI":"10.1084\/jem.20112391","volume":"209","author":"U Palendira","year":"2012","unstructured":"Palendira U et al (2012) Expansion of somatically reverted memory CD8+ T cells in patients with X-linked lymphoproliferative disease caused by selective pressure from Epstein-Barr virus. J Exp Med 209:913\u2013924","journal-title":"J Exp Med"},{"key":"316_CR172","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1111\/joim.13628","volume":"294","author":"Q Pan-Hammarstrom","year":"2023","unstructured":"Pan-Hammarstrom Q, Casanova JL (2023) Human genetic and immunological determinants of SARS-CoV-2 and Epstein-Barr virus diseases in childhood: insightful contrasts. J Intern Med 294:127\u2013144","journal-title":"J Intern Med"},{"key":"316_CR173","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1084\/jem.192.3.337","volume":"192","author":"S Parolini","year":"2000","unstructured":"Parolini S et al (2000) X-linked lymphoproliferative disease. 2B4 molecules displaying inhibitory rather than activating function are responsible for the inability of natural killer cells to kill Epstein-Barr virus-infected cells. J Exp Med 192:337\u2013346","journal-title":"J Exp Med"},{"key":"316_CR174","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1007\/s10875-016-0360-8","volume":"37","author":"M Parta","year":"2017","unstructured":"Parta M et al (2017) Resolution of multifocal Epstein-Barr virus-related smooth muscle tumor in a patient with GATA2 deficiency following hematopoietic stem cell transplantation. J Clin Immunol 37:61\u201366","journal-title":"J Clin Immunol"},{"key":"316_CR175","doi-asserted-by":"crossref","first-page":"695","DOI":"10.1084\/jem.20042432","volume":"201","author":"B Pasquier","year":"2005","unstructured":"Pasquier B et al (2005) Defective NKT cell development in mice and humans lacking the adapter SAP, the X-linked lymphoproliferative syndrome gene product. J Exp Med 201:695\u2013701","journal-title":"J Exp Med"},{"key":"316_CR176","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1016\/j.clim.2015.04.015","volume":"159","author":"T Patiroglu","year":"2015","unstructured":"Patiroglu T et al (2015) A case of XMEN syndrome presented with severe auto-immune disorders mimicking autoimmune lymphoproliferative disease. Clin Immunol 159:58\u201362","journal-title":"Clin Immunol"},{"key":"316_CR177","doi-asserted-by":"crossref","first-page":"e93739","DOI":"10.1172\/jci.insight.93739","volume":"2","author":"ML Pekalski","year":"2017","unstructured":"Pekalski ML et al (2017) Neonatal and adult recent thymic emigrants produce IL-8 and express complement receptors CR1 and CR2. JCI Insight 2:e93739","journal-title":"JCI Insight"},{"key":"316_CR178","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1016\/j.clim.2018.10.022","volume":"204","author":"D Pende","year":"2019","unstructured":"Pende D, Meazza R, Marcenaro S, Arico M, Bottino C (2019) 2B4 dysfunction in XLP1 NK cells: more than inability to control EBV infection. Clin Immunol 204:31\u201336","journal-title":"Clin Immunol"},{"key":"316_CR179","doi-asserted-by":"crossref","first-page":"1288","DOI":"10.1016\/j.jaci.2019.11.049","volume":"145","author":"A Perez-Portilla","year":"2020","unstructured":"Perez-Portilla A et al (2020) Identification of the first cases of complete CD16A deficiency: association with persistent EBV infection. J Allergy Clin Immunol 145:1288\u20131292","journal-title":"J Allergy Clin Immunol"},{"key":"316_CR180","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1016\/j.clim.2017.08.007","volume":"183","author":"CD Platt","year":"2017","unstructured":"Platt CD et al (2017) Combined immunodeficiency with EBV positive B cell lymphoma and epidermodysplasia verruciformis due to a novel homozygous mutation in RASGRP1. Clin Immunol 183:142\u2013144","journal-title":"Clin Immunol"},{"key":"316_CR227","doi-asserted-by":"crossref","unstructured":"Poli MC (2025) Human inborn errors of immunity: 2024 update on the classification from the International Union of Immunological Societies Expert Committee. J Hum Immun 1:e20250003.","DOI":"10.70962\/jhi.20250003"},{"key":"316_CR181","doi-asserted-by":"crossref","first-page":"e2116741119","DOI":"10.1073\/pnas.2116741119","volume":"119","author":"I Pratumchai","year":"2022","unstructured":"Pratumchai I et al (2022) B cell-derived IL-27 promotes control of persistent LCMV infection. Proc Natl Acad Sci USA 119:e2116741119","journal-title":"Proc Natl Acad Sci USA"},{"key":"316_CR182","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1056\/NEJM197507102930202","volume":"293","author":"AJ Provisor","year":"1975","unstructured":"Provisor AJ, Iacuone JJ, Chilcote RR, Neiburger RG, Crussi FG (1975) Acquired agammaglobulinemia after a life-threatening illness with clinical and laboratory features of infectious mononucleosis in three related male children. N Engl J Med 293:62\u201365","journal-title":"N Engl J Med"},{"key":"316_CR183","doi-asserted-by":"crossref","first-page":"e20211387","DOI":"10.1084\/jem.20211387","volume":"219","author":"A Puel","year":"2022","unstructured":"Puel A, Bastard P, Bustamante J, Casanova JL (2022) Human autoantibodies underlying infectious diseases. J Exp Med 219:e20211387","journal-title":"J Exp Med"},{"key":"316_CR184","doi-asserted-by":"crossref","first-page":"100","DOI":"10.1007\/s10875-015-0130-z","volume":"35","author":"D Punwani","year":"2015","unstructured":"Punwani D et al (2015) Coronin-1A: immune deficiency in humans and mice. J Clin Immunol 35:100\u2013107","journal-title":"J Clin Immunol"},{"key":"316_CR185","volume":"2011","author":"B Purgina","year":"2011","unstructured":"Purgina B, Rao UN, Miettinen M, Pantanowitz L (2011) AIDS-related EBV-associated smooth muscle tumors: a review of 64 published cases. Pathol Res Int 2011:561548","journal-title":"Pathol Res Int"},{"key":"316_CR186","doi-asserted-by":"crossref","first-page":"935","DOI":"10.1016\/S0140-6736(75)92004-8","volume":"1","author":"DT Purtilo","year":"1975","unstructured":"Purtilo DT, Cassel CK, Yang JP, Harper R (1975) X-linked recessive progressive combined variable immunodeficiency (Duncan\u2019s disease). Lancet 1:935\u2013940","journal-title":"Lancet"},{"key":"316_CR187","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1007\/s10875-020-00790-x","volume":"40","author":"JC Ravell","year":"2020","unstructured":"Ravell JC, Chauvin SD, He T, Lenardo M (2020a) An update on XMEN disease. J Clin Immunol 40:671\u2013681","journal-title":"J Clin Immunol"},{"key":"316_CR188","doi-asserted-by":"crossref","first-page":"507","DOI":"10.1172\/JCI131116","volume":"130","author":"JC Ravell","year":"2020","unstructured":"Ravell JC et al (2020b) Defective glycosylation and multisystem abnormalities characterize the primary immunodeficiency XMEN disease. J Clin Invest 130:507\u2013522","journal-title":"J Clin Invest"},{"key":"316_CR189","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1053\/hupa.2002.30214","volume":"33","author":"C Reyes","year":"2002","unstructured":"Reyes C, Abuzaitoun O, De Jong A, Hanson C, Langston C (2002) Epstein-Barr virus-associated smooth muscle tumors in ataxia-telangiectasia: a case report and review. Hum Pathol 33:133\u2013136","journal-title":"Hum Pathol"},{"key":"316_CR190","doi-asserted-by":"crossref","first-page":"110","DOI":"10.1038\/nature05257","volume":"444","author":"S Rigaud","year":"2006","unstructured":"Rigaud S et al (2006) XIAP deficiency in humans causes an X-linked lymphoproliferative syndrome. Nature 444:110\u2013114","journal-title":"Nature"},{"key":"316_CR191","doi-asserted-by":"crossref","first-page":"252","DOI":"10.1182\/blood-2011-01-328849","volume":"118","author":"S Rigaud","year":"2011","unstructured":"Rigaud S et al (2011) Human X-linked variable immunodeficiency caused by a hypomorphic mutation in XIAP in association with a rare polymorphism in CD40LG. Blood 118:252\u2013261","journal-title":"Blood"},{"key":"316_CR192","doi-asserted-by":"crossref","first-page":"2800","DOI":"10.1084\/jem.20190678","volume":"216","author":"R Rodriguez","year":"2019","unstructured":"Rodriguez R et al (2019) Concomitant PIK3CD and TNFRSF9 deficiencies cause chronic active Epstein-Barr virus infection of T cells. J Exp Med 216:2800\u20132818","journal-title":"J Exp Med"},{"key":"316_CR193","doi-asserted-by":"crossref","first-page":"2437","DOI":"10.1084\/jem.20160579","volume":"213","author":"R Roncagalli","year":"2016","unstructured":"Roncagalli R et al (2016) The scaffolding function of the RLTPR protein explains its essential role for CD28 co-stimulation in mouse and human T cells. J Exp Med 213:2437\u20132457","journal-title":"J Exp Med"},{"key":"316_CR194","doi-asserted-by":"crossref","first-page":"393","DOI":"10.1007\/s00428-021-03147-w","volume":"480","author":"A Saglam","year":"2022","unstructured":"Saglam A et al (2022) STK4 deficiency and EBV-associated lymphoproliferative disorders, emphasis on histomorphology, and review of literature. Virchows Arch 480:393\u2013401","journal-title":"Virchows Arch"},{"key":"316_CR195","doi-asserted-by":"crossref","first-page":"473","DOI":"10.3324\/haematol.2012.068791","volume":"98","author":"E Salzer","year":"2013","unstructured":"Salzer E et al (2013) Combined immunodeficiency with life-threatening EBV-associated lymphoproliferative disorder in patients lacking functional CD27. Haematologica 98:473\u2013478","journal-title":"Haematologica"},{"key":"316_CR196","doi-asserted-by":"crossref","first-page":"1352","DOI":"10.1038\/ni.3575","volume":"17","author":"E Salzer","year":"2016","unstructured":"Salzer E et al (2016) RASGRP1 deficiency causes immunodeficiency with impaired cytoskeletal dynamics. Nat Immunol 17:1352\u20131360","journal-title":"Nat Immunol"},{"key":"316_CR197","doi-asserted-by":"crossref","first-page":"462","DOI":"10.1038\/26683","volume":"395","author":"J Sayos","year":"1998","unstructured":"Sayos J et al (1998) The X-linked lymphoproliferative-disease gene product SAP regulates signals induced through the co-receptor SLAM. Nature 395:462\u2013469","journal-title":"Nature"},{"key":"316_CR198","doi-asserted-by":"crossref","first-page":"2400","DOI":"10.3389\/fimmu.2018.02400","volume":"9","author":"C Schipp","year":"2018","unstructured":"Schipp C et al (2018) EBV negative lymphoma and autoimmune lymphoproliferative syndrome like phenotype extend the clinical spectrum of primary immunodeficiency caused by STK4 deficiency. Front Immunol 9:2400","journal-title":"Front Immunol"},{"key":"316_CR199","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1002\/eji.201040804","volume":"41","author":"R Schneider","year":"2011","unstructured":"Schneider R, Yaneva T, Beauseigle D, El-Khoury L, Arbour N (2011) IL-27 increases the proliferation and effector functions of human naive CD8+ T lymphocytes and promotes their development into Tc1 cells. Eur J Immunol 41:47\u201359","journal-title":"Eur J Immunol"},{"key":"316_CR200","doi-asserted-by":"crossref","first-page":"14209","DOI":"10.1038\/ncomms14209","volume":"8","author":"T Schober","year":"2017","unstructured":"Schober T et al (2017) A human immunodeficiency syndrome caused by mutations in CARMIL2. Nat Commun 8:14209","journal-title":"Nat Commun"},{"key":"316_CR201","doi-asserted-by":"crossref","first-page":"E830","DOI":"10.1002\/ijc.27416","volume":"131","author":"R Scholtysik","year":"2012","unstructured":"Scholtysik R et al (2012) Recurrent deletions of the TNFSF7 and TNFSF9 genes in 19p13.3 in diffuse large B-cell and Burkitt lymphomas. Int J Cancer 131:E830\u2013E835","journal-title":"Int J Cancer"},{"key":"316_CR202","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1007\/978-3-031-59815-9_8","volume":"1448","author":"GS Schulert","year":"2024","unstructured":"Schulert GS, Zhang K (2024) Genetics of acquired cytokine storm syndromes: secondary HLH genetics. Adv Exp Med Biol 1448:103\u2013119","journal-title":"Adv Exp Med Biol"},{"key":"316_CR203","doi-asserted-by":"crossref","first-page":"434","DOI":"10.1007\/s10875-017-0396-4","volume":"37","author":"Y Sekinaka","year":"2017","unstructured":"Sekinaka Y et al (2017) Common variable immunodeficiency caused by FANC mutations. J Clin Immunol 37:434\u2013444","journal-title":"J Clin Immunol"},{"key":"316_CR204","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1159\/000076437","volume":"41","author":"Y Seko","year":"2004","unstructured":"Seko Y et al (2004) Expression of costimulatory molecules (4-1BBL and Fas) and major histocompatibility class I chain-related A (MICA) in aortic tissue with Takayasu\u2019s arteritis. J Vasc Res 41:84\u201390","journal-title":"J Vasc Res"},{"key":"316_CR205","doi-asserted-by":"crossref","first-page":"655","DOI":"10.1182\/blood-2014-03-564930","volume":"124","author":"NK Serwas","year":"2014","unstructured":"Serwas NK et al (2014) Identification of ITK deficiency as a novel genetic cause of idiopathic CD4+ T-cell lymphopenia. Blood 124:655\u2013657","journal-title":"Blood"},{"key":"316_CR206","doi-asserted-by":"crossref","first-page":"2444","DOI":"10.1182\/bloodadvances.2021006367","volume":"6","author":"T Shahin","year":"2022","unstructured":"Shahin T et al (2022) Identification of germline monoallelic mutations in IKZF2 in patients with immune dysregulation. Blood Adv 6:2444\u20132451","journal-title":"Blood Adv"},{"key":"316_CR207","doi-asserted-by":"crossref","first-page":"713","DOI":"10.3389\/fonc.2019.00713","volume":"9","author":"C Shannon-Lowe","year":"2019","unstructured":"Shannon-Lowe C, Rickinson A (2019) The global landscape of EBV-associated tumors. Front Oncol 9:713","journal-title":"Front Oncol"},{"key":"316_CR208","doi-asserted-by":"crossref","DOI":"10.1002\/cti2.1448","volume":"12","author":"K Shen","year":"2023","unstructured":"Shen K et al (2023) CD137 deficiency because of two novel biallelic TNFRSF9 mutations in a patient presenting with severe EBV-associated lymphoproliferative disease. Clin Transl Immunol 12:e1448","journal-title":"Clin Transl Immunol"},{"key":"316_CR209","doi-asserted-by":"crossref","DOI":"10.1097\/MD.0000000000008852","volume":"96","author":"R Sherkat","year":"2017","unstructured":"Sherkat R et al (2017) EBV lymphoproliferative-associated disease and primary cardiac T-cell lymphoma in a STK4 deficient patient: A case report. Medicine (Baltimore) 96:e8852","journal-title":"Medicine (Baltimore)"},{"key":"316_CR210","first-page":"150","volume":"0","author":"T Shigemura","year":"2024","unstructured":"Shigemura T, Kobayashi N, Uehara T, Nakazawa Y (2024) Fatal CAEBV-associated vasculitis in ICF syndrome type 2. J Investig Allergol Clin Immunol 0:150","journal-title":"J Investig Allergol Clin Immunol"},{"key":"316_CR211","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1016\/j.clim.2008.11.002","volume":"131","author":"LR Shiow","year":"2009","unstructured":"Shiow LR et al (2009) Severe combined immunodeficiency (SCID) and attention deficit hyperactivity disorder (ADHD) associated with a Coronin-1A mutation and a chromosome 16p11.2 deletion. Clin Immunol 131:24\u201330","journal-title":"Clin Immunol"},{"key":"316_CR212","first-page":"2976","volume":"119","author":"AL Snow","year":"2009","unstructured":"Snow AL et al (2009) Restimulation-induced apoptosis of T cells is impaired in patients with X-linked lymphoproliferative disease caused by SAP deficiency. J Clin Invest 119:2976\u20132989","journal-title":"J Clin Invest"},{"key":"316_CR213","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1007\/978-981-32-9717-3_3","volume":"1189","author":"T So","year":"2019","unstructured":"So T, Ishii N (2019) The TNF-TNFR family of co-signal molecules. Adv Exp Med Biol 1189:53\u201384","journal-title":"Adv Exp Med Biol"},{"key":"316_CR214","doi-asserted-by":"crossref","first-page":"699","DOI":"10.1007\/s10875-018-0533-8","volume":"38","author":"I Somekh","year":"2018","unstructured":"Somekh I et al (2018a) Novel mutations in RASGRP1 are associated with immunodeficiency, immune dysregulation, and EBV-induced lymphoma. J Clin Immunol 38:699\u2013710","journal-title":"J Clin Immunol"},{"key":"316_CR215","doi-asserted-by":"crossref","first-page":"711","DOI":"10.1007\/s10875-018-0541-8","volume":"38","author":"I Somekh","year":"2018","unstructured":"Somekh I et al (2018b) Correction to: novel mutations in RASGRP1 are associated with immunodeficiency, immune dysregulation, and EBV-induced lymphoma. J Clin Immunol 38:711","journal-title":"J Clin Immunol"},{"key":"316_CR216","doi-asserted-by":"crossref","first-page":"1510","DOI":"10.1182\/blood.2019000644","volume":"134","author":"I Somekh","year":"2019","unstructured":"Somekh I et al (2019) CD137 deficiency causes immune dysregulation with predisposition to lymphomagenesis. Blood 134:1510\u20131516","journal-title":"Blood"},{"key":"316_CR217","doi-asserted-by":"crossref","first-page":"1982","DOI":"10.1038\/s41467-024-45805-y","volume":"15","author":"C Soudais","year":"2024","unstructured":"Soudais C et al (2024) Inactivation of cytidine triphosphate synthase 1 prevents fatal auto-immunity in mice. Nat Commun 15:1982","journal-title":"Nat Commun"},{"key":"316_CR218","doi-asserted-by":"crossref","first-page":"472","DOI":"10.3324\/haematol.2010.033910","volume":"96","author":"P Stepensky","year":"2011","unstructured":"Stepensky P et al (2011) IL-2-inducible T-cell kinase deficiency: clinical presentation and therapeutic approach. Haematologica 96:472\u2013476","journal-title":"Haematologica"},{"key":"316_CR219","doi-asserted-by":"crossref","first-page":"486","DOI":"10.1002\/ccr3.880","volume":"5","author":"T Stevens","year":"2017","unstructured":"Stevens T, van der Werff Ten Bosch J, De Rademaeker M, Van Den Bogaert A, van den Akker M (2017) Risk of malignancy in 22q11.2 deletion syndrome. Clin Case Rep 5:486\u2013490","journal-title":"Clin Case Rep"},{"key":"316_CR220","doi-asserted-by":"crossref","first-page":"871","DOI":"10.1007\/s10875-014-0074-8","volume":"34","author":"A Stray-Pedersen","year":"2014","unstructured":"Stray-Pedersen A et al (2014) Compound heterozygous CORO1A mutations in siblings with a mucocutaneous-immunodeficiency syndrome of epidermodysplasia verruciformis-HPV, molluscum contagiosum and granulomatous tuberculoid leprosy. J Clin Immunol 34:871\u2013890","journal-title":"J Clin Immunol"},{"key":"316_CR221","doi-asserted-by":"crossref","DOI":"10.1111\/tid.13010","volume":"21","author":"RJ Stubbins","year":"2019","unstructured":"Stubbins RJ et al (2019) Epstein-Barr virus associated smooth muscle tumors in solid organ transplant recipients: incidence over 31 years at a single institution and review of the literature. Transpl Infect Dis 21:e13010","journal-title":"Transpl Infect Dis"},{"key":"316_CR222","doi-asserted-by":"crossref","first-page":"985","DOI":"10.1086\/423285","volume":"190","author":"N Sugaya","year":"2004","unstructured":"Sugaya N et al (2004) Quantitative analysis of Epstein-Barr virus (EBV)-specific CD8+ T cells in patients with chronic active EBV infection. J Infect Dis 190:985\u2013988","journal-title":"J Infect Dis"},{"key":"316_CR223","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1007\/s10875-024-01701-0","volume":"44","author":"T Suzuki","year":"2024","unstructured":"Suzuki T et al (2024) Single-cell transcriptomic analysis of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis. J Clin Immunol 44:103","journal-title":"J Clin Immunol"},{"key":"316_CR224","doi-asserted-by":"crossref","first-page":"772","DOI":"10.1007\/s10875-014-0083-7","volume":"34","author":"SG Tangye","year":"2014","unstructured":"Tangye SG (2014) XLP: clinical features and molecular etiology due to mutations in SH2D1A encoding SAP. J Clin Immunol 34:772\u2013779","journal-title":"J Clin Immunol"},{"key":"316_CR225","doi-asserted-by":"crossref","first-page":"644","DOI":"10.1182\/blood.2019000928","volume":"135","author":"SG Tangye","year":"2020","unstructured":"Tangye SG, Latour S (2020) Primary immunodeficiencies reveal the molecular requirements for effective host defense against EBV infection. Blood 135:644\u2013655","journal-title":"Blood"},{"key":"316_CR226","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1084\/jem.20161846","volume":"214","author":"SG Tangye","year":"2017","unstructured":"Tangye SG, Palendira U, Edwards ES (2017) Human immunity against EBV-lessons from the clinic. J Exp Med 214:269\u2013283","journal-title":"J Exp Med"},{"key":"316_CR228","doi-asserted-by":"crossref","first-page":"15","DOI":"10.3389\/fped.2019.00015","volume":"7","author":"K Tanita","year":"2019","unstructured":"Tanita K et al (2019) Epstein-Barr virus-associated gammadelta T-cell lymphoproliferative disorder associated with hypomorphic IL2RG mutation. Front Pediatr 7:15","journal-title":"Front Pediatr"},{"key":"316_CR229","doi-asserted-by":"crossref","first-page":"3060","DOI":"10.3389\/fimmu.2018.03060","volume":"9","author":"M Tatfi","year":"2018","unstructured":"Tatfi M, Hermine O, Suarez F (2018) Epstein-Barr virus (EBV)-related lymphoproliferative disorders in ataxia telangiectasia: does ATM regulate EBV life cycle? Front Immunol 9:3060","journal-title":"Front Immunol"},{"key":"316_CR230","doi-asserted-by":"crossref","first-page":"787","DOI":"10.1146\/annurev-immunol-032414-112326","volume":"33","author":"GS Taylor","year":"2015","unstructured":"Taylor GS, Long HM, Brooks JM, Rickinson AB, Hislop AD (2015) The immunology of Epstein-Barr virus-induced disease. Annu Rev Immunol 33:787\u2013821","journal-title":"Annu Rev Immunol"},{"key":"316_CR231","first-page":"151","volume":"390","author":"DA Thorley-Lawson","year":"2015","unstructured":"Thorley-Lawson DA (2015) EBV persistence--introducing the virus. Curr Top Microbiol Immunol 390:151\u2013209","journal-title":"Curr Top Microbiol Immunol"},{"key":"316_CR232","doi-asserted-by":"crossref","first-page":"976","DOI":"10.1042\/bst0210976","volume":"21","author":"DA Thorley-Lawson","year":"1993","unstructured":"Thorley-Lawson DA, Ianelli C, Klaman LD, Staunton D, Yokoyama S (1993) Function of CD48 and its regulation by Epstein-Barr virus. Biochem Soc Trans 21:976\u2013980","journal-title":"Biochem Soc Trans"},{"key":"316_CR233","doi-asserted-by":"crossref","first-page":"955","DOI":"10.1016\/S1470-2045(06)70943-3","volume":"7","author":"HC Toh","year":"2006","unstructured":"Toh HC et al (2006) Use of sirolimus for Epstein-Barr virus-positive smooth-muscle tumour. Lancet Oncol 7:955\u2013957","journal-title":"Lancet Oncol"},{"key":"316_CR234","doi-asserted-by":"crossref","DOI":"10.1002\/pbc.29026","volume":"68","author":"MP Triebwasser","year":"2021","unstructured":"Triebwasser MP et al (2021) Combined use of emapalumab and ruxolitinib in a patient with refractory hemophagocytic lymphohistiocytosis was safe and effective. Pediatr Blood Cancer 68:e29026","journal-title":"Pediatr Blood Cancer"},{"key":"316_CR235","doi-asserted-by":"crossref","first-page":"5352","DOI":"10.4049\/jimmunol.0802809","volume":"182","author":"KP van Gisbergen","year":"2009","unstructured":"van Gisbergen KP et al (2009) Protective CD8 T cell memory is impaired during chronic CD70-driven costimulation. J Immunol 182:5352\u20135362","journal-title":"J Immunol"},{"key":"316_CR236","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1016\/j.immuni.2011.04.020","volume":"35","author":"KP van Gisbergen","year":"2011","unstructured":"van Gisbergen KP et al (2011) The costimulatory molecule CD27 maintains clonally diverse CD8(+) T cell responses of low antigen affinity to protect against viral variants. Immunity 35:97\u2013108","journal-title":"Immunity"},{"key":"316_CR237","doi-asserted-by":"crossref","first-page":"787","DOI":"10.1016\/j.jaci.2011.11.013","volume":"129","author":"JM van Montfrans","year":"2012","unstructured":"van Montfrans JM et al (2012) CD27 deficiency is associated with combined immunodeficiency and persistent symptomatic EBV viremia. J Allergy Clin Immunol 129:787\u2013793 e786","journal-title":"J Allergy Clin Immunol"},{"key":"316_CR238","volume":"36","author":"NW Wah","year":"2023","unstructured":"Wah NW et al (2023) Clinicopathologic and molecular characteristics of Epstein-Barr virus-associated smooth muscle tumor compared with those of leiomyoma and leiomyosarcoma. Mod Pathol 36:100127","journal-title":"Mod Pathol"},{"key":"316_CR239","doi-asserted-by":"crossref","first-page":"743","DOI":"10.1016\/j.jaci.2020.06.019","volume":"147","author":"JG Wallace","year":"2021","unstructured":"Wallace JG et al (2021) ITK deficiency presenting as autoimmune lymphoproliferative syndrome. J Allergy Clin Immunol 147:743\u2013745 e741","journal-title":"J Allergy Clin Immunol"},{"key":"316_CR240","doi-asserted-by":"crossref","first-page":"2413","DOI":"10.1084\/jem.20160576","volume":"213","author":"Y Wang","year":"2016","unstructured":"Wang Y et al (2016) Dual T cell- and B cell-intrinsic deficiency in humans with biallelic RLTPR mutations. J Exp Med 213:2413\u20132435","journal-title":"J Exp Med"},{"key":"316_CR241","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1182\/blood.2023021074","volume":"143","author":"J Wang","year":"2024","unstructured":"Wang J et al (2024) Chronic active Epstein-Barr virus disease originates from infected hematopoietic stem cells. Blood 143:32\u201341","journal-title":"Blood"},{"key":"316_CR242","doi-asserted-by":"crossref","first-page":"188","DOI":"10.15252\/emmm.201708292","volume":"10","author":"S Winter","year":"2018","unstructured":"Winter S et al (2018) Loss of RASGRP1 in humans impairs T-cell expansion leading to Epstein-Barr virus susceptibility. EMBO Mol Med 10:188\u2013199","journal-title":"EMBO Mol Med"},{"key":"316_CR243","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1182\/blood-2006-11-056168","volume":"110","author":"M Wolfl","year":"2007","unstructured":"Wolfl M et al (2007) Activation-induced expression of CD137 permits detection, isolation, and expansion of the full repertoire of CD8+ T cells responding to antigen without requiring knowledge of epitope specificities. Blood 110:201\u2013210","journal-title":"Blood"},{"key":"316_CR244","doi-asserted-by":"crossref","DOI":"10.1128\/jvi.00394-22","volume":"96","author":"W Wongwiwat","year":"2022","unstructured":"Wongwiwat W et al (2022) Epstein-Barr virus genome deletions in Epstein-Barr virus-positive T\/NK cell lymphoproliferative diseases. J Virol 96:e0039422","journal-title":"J Virol"},{"key":"316_CR245","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1111\/imr.12086","volume":"255","author":"ME Wortzman","year":"2013","unstructured":"Wortzman ME, Clouthier DL, McPherson AJ, Lin GH, Watts TH (2013) The contextual role of TNFR family members in CD8(+) T-cell control of viral infections. Immunol Rev 255:125\u2013148","journal-title":"Immunol Rev"},{"key":"316_CR246","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1016\/j.coi.2015.11.003","volume":"38","author":"N Wu","year":"2016","unstructured":"Wu N, Veillette A (2016) SLAM family receptors in normal immunity and immune pathologies. Curr Opin Immunol 38:45\u201351","journal-title":"Curr Opin Immunol"},{"key":"316_CR247","doi-asserted-by":"crossref","first-page":"387","DOI":"10.1038\/ni.3369","volume":"17","author":"N Wu","year":"2016","unstructured":"Wu N et al (2016) A hematopoietic cell-driven mechanism involving SLAMF6 receptor, SAP adaptors and SHP-1 phosphatase regulates NK cell education. Nat Immunol 17:387\u2013396","journal-title":"Nat Immunol"},{"key":"316_CR248","doi-asserted-by":"crossref","first-page":"e261993","DOI":"10.1136\/bcr-2024-261993","volume":"17","author":"KR Xu","year":"2024","unstructured":"Xu KR, Shah S, Rohr B (2024) Systemic hydroa vacciniforme lymphoproliferative disorder in a patient with chronic active EBV infection. BMJ Case Rep 17:e261993","journal-title":"BMJ Case Rep"},{"key":"316_CR249","doi-asserted-by":"crossref","first-page":"1796","DOI":"10.1016\/j.celrep.2014.05.008","volume":"7","author":"M Yabal","year":"2014","unstructured":"Yabal M et al (2014) XIAP restricts TNF- and RIP3-dependent cell death and inflammasome activation. Cell Rep 7:1796\u20131808","journal-title":"Cell Rep"},{"key":"316_CR250","doi-asserted-by":"crossref","first-page":"780","DOI":"10.1002\/ajh.26544","volume":"97","author":"M Yamamoto","year":"2022","unstructured":"Yamamoto M et al (2022) Registry data analysis of hematopoietic stem cell transplantation on systemic chronic active Epstein-Barr virus infection patients in Japan. Am J Hematol 97:780\u2013790","journal-title":"Am J Hematol"},{"key":"316_CR251","doi-asserted-by":"crossref","first-page":"244","DOI":"10.1007\/s10875-015-0144-6","volume":"35","author":"X Yang","year":"2015","unstructured":"Yang X et al (2015) A female patient with incomplete hemophagocytic lymphohistiocytosis caused by a heterozygous XIAP mutation associated with non-random X-chromosome inactivation skewed towards the wild-type XIAP allele. J Clin Immunol 35:244\u2013248","journal-title":"J Clin Immunol"},{"key":"316_CR252","doi-asserted-by":"crossref","first-page":"879","DOI":"10.1016\/j.jaci.2015.08.020","volume":"137","author":"CS Yee","year":"2016","unstructured":"Yee CS et al (2016) Recurrent viral infections associated with a homozygous CORO1A mutation that disrupts oligomerization and cytoskeletal association. J Allergy Clin Immunol 137:879\u2013888 e872","journal-title":"J Allergy Clin Immunol"},{"key":"316_CR253","doi-asserted-by":"crossref","first-page":"172","DOI":"10.1007\/s10875-024-01780-z","volume":"44","author":"W Ying","year":"2024","unstructured":"Ying W et al (2024) Epidermodysplasia Verruciformis and Vdelta2 gammadelta T-cell expansion in STK4 deficiency. J Clin Immunol 44:172","journal-title":"J Clin Immunol"},{"key":"316_CR254","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1146\/annurev-immunol-032414-112134","volume":"33","author":"H Yoshida","year":"2015","unstructured":"Yoshida H, Hunter CA (2015) The immunobiology of interleukin-27. Annu Rev Immunol 33:417\u2013443","journal-title":"Annu Rev Immunol"},{"key":"316_CR255","doi-asserted-by":"crossref","first-page":"1938","DOI":"10.1093\/cid\/ciy942","volume":"68","author":"L Youssefian","year":"2019","unstructured":"Youssefian L et al (2019) Inherited interleukin 2-inducible T-cell (ITK) kinase deficiency in siblings with epidermodysplasia Verruciformis and Hodgkin lymphoma. Clin Infect Dis 68:1938\u20131941","journal-title":"Clin Infect Dis"},{"key":"316_CR256","doi-asserted-by":"crossref","first-page":"2216","DOI":"10.3389\/fimmu.2019.02216","volume":"10","author":"Q Zeng","year":"2019","unstructured":"Zeng Q, Zhou Y, Schwarz H (2019) CD137L-DCs, potent immune-stimulators-history, characteristics, and perspectives. Front Immunol 10:2216","journal-title":"Front Immunol"},{"key":"316_CR257","doi-asserted-by":"crossref","first-page":"1003","DOI":"10.1016\/j.immuni.2012.05.017","volume":"36","author":"F Zhao","year":"2012","unstructured":"Zhao F, Cannons JL, Dutta M, Griffiths GM, Schwartzberg PL (2012) Positive and negative signaling through SLAM receptors regulate synapse organization and thresholds of cytolysis. Immunity 36:1003\u20131016","journal-title":"Immunity"},{"key":"316_CR258","volume":"96","author":"L Zhong","year":"2017","unstructured":"Zhong L et al (2017) Chronic active Epstein-Barr virus infection as the initial symptom in a Janus kinase 3 deficiency child: case report and literature review. Medicine (Baltimore) 96:e7989","journal-title":"Medicine (Baltimore)"}],"container-title":["Current Topics in Microbiology and Immunology"],"original-title":[],"link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/82_2025_316","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,12]],"date-time":"2025-08-12T15:18:27Z","timestamp":1755011907000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/82_2025_316"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025]]},"references-count":260,"URL":"https:\/\/doi.org\/10.1007\/82_2025_316","relation":{},"ISSN":["0070-217X","2196-9965"],"issn-type":[{"value":"0070-217X","type":"print"},{"value":"2196-9965","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025]]},"assertion":[{"value":"13 August 2025","order":1,"name":"first_online","label":"First Online","group":{"name":"ChapterHistory","label":"Chapter History"}}]}}