{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,4]],"date-time":"2026-11-04T17:18:04Z","timestamp":1793812684154,"version":"4.6.1"},"reference-count":24,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2025,2,20]],"date-time":"2025-02-20T00:00:00Z","timestamp":1740009600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0"},{"start":{"date-parts":[[2025,2,20]],"date-time":"2025-02-20T00:00:00Z","timestamp":1740009600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Clin Immunol"],"published-print":{"date-parts":[[2025,12]]},"DOI":"10.1007\/s10875-025-01871-5","type":"journal-article","created":{"date-parts":[[2025,2,20]],"date-time":"2025-02-20T08:13:46Z","timestamp":1740039226000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Bone Marrow CD8\u2009+\u2009Abundance Inversely Correlates with Progressive Marrow Fibrosis and Myelodysplastic Evolution in GATA2 Deficiency: Case Report"],"prefix":"10.1007","volume":"45","author":[{"given":"Francesca","family":"Vendemini","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samuele","family":"Roncareggi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincenzo","family":"L\u2019Imperio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabiola","family":"Guerra","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Federica","family":"Mottadelli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Chiarini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Oscar","family":"Maglia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simona","family":"Sala","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Grazia","family":"Fazio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rocco","family":"Piazza","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sonia","family":"Bonanomi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Biondi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"Saettini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,2,20]]},"reference":[{"issue":"8","key":"1871_CR1","doi-asserted-by":"publisher","first-page":"2192","DOI":"10.1007\/s10875-023-01583-8","volume":"43","author":"S Roncareggi","year":"2023","unstructured":"Roncareggi S, Girardi K, Fioredda F, Pedace L, Arcuri L, Badolato R, et al. A Nationwide Study of GATA2 Deficiency in Italy reveals novel symptoms and genotype-phenotype Association. J Clin Immunol. 2023;43(8):2192\u2013207. https:\/\/doi.org\/10.1007\/s10875-023-01583-8.","journal-title":"J Clin Immunol"},{"issue":"11","key":"1871_CR2","doi-asserted-by":"publisher","first-page":"1387","DOI":"10.1182\/blood-2015-09-669937","volume":"127","author":"MW Wlodarski","year":"2016","unstructured":"Wlodarski MW, Hirabayashi S, Pastor V, Star\u00fd J, Hasle H 4, Masetti R, et al. Prevalence, clinical characteristics, and prognosis of GATA2-related myelodysplastic syndromes in children and adolescents. Blood. 2016;127(11):1387\u201397.","journal-title":"Blood"},{"key":"1871_CR3","doi-asserted-by":"publisher","unstructured":"Rodriguez-Sevilla JJ, Colla S. T cell dysfunctions in myelodysplastic syndromes. Blood 2024 Jan. 18:blood2023023166. https:\/\/doi.org\/10.1182\/blood.2023023166.","DOI":"10.1182\/blood.2023023166"},{"key":"1871_CR4","doi-asserted-by":"publisher","first-page":"802","DOI":"10.3389\/fimmu.2017.00802","volume":"8","author":"R Ruiz-Garc\u00eda","year":"2017","unstructured":"Ruiz-Garc\u00eda R, Rodr\u00edguez-Vigil C, Marco FM, Gallego-Bustos F, Castro-Panete MJ, Diez-Alonso L, et al. Acquired senescent T-Cell phenotype correlates with clinical severity in GATA binding protein 2-Deficient patients. Front Immunol. 2017;8:802. https:\/\/doi.org\/10.3389\/fimmu.2017.00802.","journal-title":"Front Immunol"},{"issue":"5","key":"1871_CR5","doi-asserted-by":"publisher","first-page":"510","DOI":"10.1080\/08880018.2020.1863536","volume":"38","author":"F Saettini","year":"2021","unstructured":"Saettini F, Coliva T, Vendemini F, Moratto D, Savoldi G, Borlenghi E, et al. When to suspect GATA2 deficiency in pediatric patients: from complete blood count to diagnosis. Pediatr Hematol Oncol. 2021;38(5):510\u20134 Epub 2021 Mar 16. PMID: 33726626.","journal-title":"Pediatr Hematol Oncol"},{"key":"1871_CR6","doi-asserted-by":"publisher","first-page":"1031687","DOI":"10.3389\/fped.2022.1031687","volume":"10","author":"F Guerra","year":"2022","unstructured":"Guerra F, L\u2019Imperio V, Bonanomi S, Spinelli M, Coliva TA, Dell\u2019Acqua F, et al. Pediatric immune myelofibrosis (PedIMF) as a novel and distinct clinical pathological entity. Front Pediatr. 2022;10:1031687. https:\/\/doi.org\/10.3389\/fped.2022.1031687. PMID: 36419910; PMCID: PMC9676962.","journal-title":"Front Pediatr"},{"issue":"9","key":"1871_CR7","doi-asserted-by":"publisher","first-page":"2241","DOI":"10.1038\/s41375-019-0496-7","volume":"33","author":"M Br\u00fcggemann","year":"2019","unstructured":"Br\u00fcggemann M, Kotrov\u00e1 M, Knecht H, Bartram J, Boudjogrha M, Bystry V, et al. Standardized next-generation sequencing of immunoglobulin and T-cell receptor gene recombinations for MRD marker identification in acute lymphoblastic leukaemia; a EuroClonality-NGS validation study. Leukemia. 2019;33(9):2241\u201353. https:\/\/doi.org\/10.1038\/s41375-019-0496-7.","journal-title":"Leukemia"},{"issue":"1","key":"1871_CR8","doi-asserted-by":"publisher","first-page":"16878","DOI":"10.1038\/s41598-017-17204-5","volume":"7","author":"P Bankhead","year":"2017","unstructured":"Bankhead P, Loughrey MB, Fern\u00e1ndez JA, Dombrowski Y, McArt DG, Dunne PD, et al. QuPath: open source software for digital pathology image analysis. Sci Rep. 2017;7(1):16878. https:\/\/doi.org\/10.1038\/s41598-017-17204-5. PMID: 29203879; PMCID: PMC5715110.","journal-title":"Sci Rep"},{"key":"1871_CR9","first-page":"265","volume":"2018","author":"U Schmidt","year":"2018","unstructured":"Schmidt U, Weigert M, Broaddus C, Myers G. Cell detection with Star-Convex polygons. Med Image Comput Comput Assist Intervention \u2013 MICCAI. 2018;2018:265\u201373.","journal-title":"Med Image Comput Comput Assist Intervention \u2013 MICCAI"},{"issue":"7","key":"1871_CR10","doi-asserted-by":"publisher","first-page":"e3694","DOI":"10.1002\/dmrr.3694","volume":"39","author":"C Grasselli","year":"2023","unstructured":"Grasselli C, Bombelli S, D\u2019Esposito V, Di Tolla MF, L\u2019Imperio V, Rocchio F, et al. The therapeutic potential of an allosteric non-competitive CXCR1\/2 antagonist for diabetic nephropathy. Diabetes Metab Res Rev. 2023;39(7):e3694. https:\/\/doi.org\/10.1002\/dmrr.3694. Epub 2023 Jul 20. PMID: 37470287.","journal-title":"Diabetes Metab Res Rev"},{"issue":"20","key":"1871_CR11","doi-asserted-by":"publisher","first-page":"2391","DOI":"10.1182\/blood-2016-03-643544","volume":"127","author":"DA Arber","year":"2016","unstructured":"Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Le Beau MM, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016;127(20):2391\u2013405. https:\/\/doi.org\/10.1182\/blood-2016-03-643544.","journal-title":"Blood"},{"issue":"13","key":"1871_CR12","doi-asserted-by":"publisher","first-page":"1524","DOI":"10.1182\/blood.2022017764","volume":"141","author":"KR Calvo","year":"2023","unstructured":"Calvo KR, Hickstein DD. The spectrum of GATA2 deficiency syndrome. Blood. 2023;141(13):1524\u201332. https:\/\/doi.org\/10.1182\/blood.2022017764.","journal-title":"Blood"},{"issue":"3","key":"1871_CR13","doi-asserted-by":"publisher","first-page":"793","DOI":"10.1182\/bloodadvances.2021005065","volume":"6","author":"RR West","year":"2022","unstructured":"West RR, Calvo KR, Embree LJ, Wang W, Tuschong LM, Bauer TR, et al. ASXL1 and STAG2 are common mutations in GATA2 deficiency patients with bone marrow disease and myelodysplastic syndrome. Blood Adv. 2022;6(3):793\u2013807.","journal-title":"Blood Adv"},{"issue":"6","key":"1871_CR14","doi-asserted-by":"publisher","first-page":"1515","DOI":"10.3324\/haematol.2022.282250","volume":"108","author":"L Largeaud","year":"2023","unstructured":"Largeaud L, Collin M, Monselet N, Vergez F, Fregona V, Larcher L, et al. Somatic genetic alterations predict hematological progression in GATA2 deficiency. Haematologica. 2023;108(6):1515\u201329. https:\/\/doi.org\/10.3324\/haematol.2022.282250. PMID: 36727400; PMCID: PMC10230419.","journal-title":"Haematologica"},{"issue":"3","key":"1871_CR15","doi-asserted-by":"publisher","first-page":"299","DOI":"10.3343\/alm.2022.42.3.299","volume":"42","author":"AG Jain","year":"2022","unstructured":"Jain AG, Zhang L, Bennett JM, Komrokji R. Myelodysplastic syndromes with bone marrow fibrosis: an update. Ann Lab Med. 2022;42(3):299\u2013305. https:\/\/doi.org\/10.3343\/alm.2022.42.3.299.","journal-title":"Ann Lab Med"},{"issue":"1","key":"1871_CR16","doi-asserted-by":"publisher","first-page":"56","DOI":"10.1182\/blood-2014-06-580340","volume":"125","author":"KA Ganapathi","year":"2015","unstructured":"Ganapathi KA, Townsley DM, Hsu AP, Arthur DC, Zerbe CS, Cuellar-Rodriguez J, et al. GATA2 deficiency-associated bone marrow disorder differs from idiopathic aplastic anemia. Blood. 2015;125(1):56\u201370. https:\/\/doi.org\/10.1182\/blood-2014-06-580340.","journal-title":"Blood"},{"issue":"8","key":"1871_CR17","doi-asserted-by":"publisher","first-page":"1221","DOI":"10.3324\/haematol.2011.041152","volume":"96","author":"KR Calvo","year":"2011","unstructured":"Calvo KR, Vinh DC, Maric I, Wang W, Noel P, Stetler-Stevenson M, et al. Myelodysplasia in autosomal dominant and sporadic monocytopenia immunodeficiency syndrome: diagnostic features and clinical implications. Haematologica. 2011;96(8):1221\u20135. https:\/\/doi.org\/10.3324\/haematol.2011.041152.","journal-title":"Haematologica"},{"issue":"11","key":"1871_CR18","doi-asserted-by":"publisher","first-page":"1200","DOI":"10.1182\/blood.2022015850","volume":"140","author":"DA Arber","year":"2022","unstructured":"Arber DA, Orazi A, Hasserjian RP, Borowitz MJ, Calvo KR, Kvasnicka HM, et al. International Consensus classification of myeloid neoplasms and Acute Leukemias: integrating morphologic, clinical, and genomic data. Blood. 2022;140(11):1200\u201328. https:\/\/doi.org\/10.1182\/blood.2022015850. PMID: 35767897; PMCID: PMC9479031.","journal-title":"Blood"},{"issue":"5","key":"1871_CR19","doi-asserted-by":"publisher","first-page":"957","DOI":"10.1038\/s41375-023-01886-0","volume":"37","author":"N Maslah","year":"2023","unstructured":"Maslah N, Benajiba L, Giraudier S, Kiladjian JJ, Cassinat B. Clonal architecture evolution in Myeloproliferative neoplasms: from a driver mutation to a complex heterogeneous mutational and phenotypic landscape. Leukemia. 2023;37(5):957\u201363. https:\/\/doi.org\/10.1038\/s41375-023-01886-0. Epub 2023 Mar 31. PMID: 37002477; PMCID: PMC10169637.","journal-title":"Leukemia"},{"issue":"3","key":"1871_CR20","doi-asserted-by":"publisher","first-page":"791","DOI":"10.1182\/bloodadvances.2020003401","volume":"5","author":"CV R\u00fctsche","year":"2021","unstructured":"R\u00fctsche CV, Haralambieva E, Lysenko V, Balabanov S, Theocharides APA. A patient with a germline GATA2 mutation and primary myelofibrosis. Blood Adv. 2021;5(3):791\u20135. https:\/\/doi.org\/10.1182\/bloodadvances.2020003401.","journal-title":"Blood Adv"},{"issue":"9","key":"1871_CR21","doi-asserted-by":"publisher","first-page":"2384","DOI":"10.3324\/haematol.2020.249441","volume":"106","author":"I Veletic","year":"2021","unstructured":"Veletic I, Prijic S, Manshouri T, Nogueras-Gonzalez GM, Verstovsek S, Estrov Z. Altered T-cell subset repertoire affects treatment outcome of patients with myelofibrosis. Haematologica. 2021;106(9):2384\u201396. https:\/\/doi.org\/10.3324\/haematol.2020.249441.","journal-title":"Haematologica"},{"issue":"16","key":"1871_CR22","doi-asserted-by":"publisher","first-page":"4373","DOI":"10.1016\/j.cell.2024.06.036","volume":"187","author":"AR Cillo","year":"2024","unstructured":"Cillo AR, Cardello C, Shan F, Karapetyan L, Kunning S, Sander C, Rush E, Karunamurthy A, Massa RC, Rohatgi A, Workman CJ, Kirkwood JM, Bruno TC, Vignali DAA. Blockade of LAG-3 and PD-1 leads to co-expression of cytotoxic and exhaustion gene modules in CD8 + T cells to promote antitumor immunity. Cell. 2024;187(16):4373-e438815. https:\/\/doi.org\/10.1016\/j.cell.2024.06.036. PMID: 39121849; PMCID: PMC11346583.","journal-title":"Cell"},{"key":"1871_CR23","doi-asserted-by":"publisher","unstructured":"Baldwin JG, Heuser-Loy C, Saha T, Schelker RC, Slavkovic-Lukic D, Strieder N, Hernandez-Lopez I, Rana N, Barden M, Mastrogiovanni F, Mart\u00edn-Santos A, Raimondi A, Brohawn P, Higgs BW, Gebhard C, Kapoor V, Telford WG, Gautam S, Xydia M, Beckhove P, Frischholz S, Schober K, Kontarakis Z, Corn JE, Iannacone M, Inverso D, Rehli M, Fioravanti J, Sengupta S, Gattinoni L. Intercellular nanotube-mediated mitochondrial transfer enhances T cell metabolic fitness and antitumor efficacy. Cell 2024 Sep. 12(24):S0092-8674. https:\/\/doi.org\/10.1016\/j.cell.2024.08.029. Epub ahead of print. PMID: 39276774.","DOI":"10.1016\/j.cell.2024.08.029"},{"issue":"9","key":"1871_CR24","doi-asserted-by":"publisher","first-page":"2316","DOI":"10.3324\/haematol.2022.279437","volume":"108","author":"I Abramovich","year":"2023","unstructured":"Abramovich I, Giladi A, Amit I, Mulet-Lazaro R, Delwel R, Gr\u00f6schel S, Levin-Zaidman S, Dezorella N, Holdengreber V, Rao TN, Yacobovich J, Steinberg-Shemer O, Huang QH, Tan Y, Chen SJ, Izraeli S, Birger Y. Cellular and metabolic characteristics of pre-leukemic hematopoietic progenitors with GATA2 haploinsufficiency. Haematologica. 2023;108(9):2316\u201330. https:\/\/doi.org\/10.3324\/haematol.2022.279437. PMID: 36475518; PMCID: PMC10483369.","journal-title":"Haematologica"}],"container-title":["Journal of Clinical Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10875-025-01871-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s10875-025-01871-5","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10875-025-01871-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,27]],"date-time":"2026-04-27T15:44:56Z","timestamp":1777304696000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s10875-025-01871-5"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,2,20]]},"references-count":24,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2025,12]]}},"alternative-id":["1871"],"URL":"https:\/\/doi.org\/10.1007\/s10875-025-01871-5","relation":{},"ISSN":["0271-9142","1573-2592"],"issn-type":[{"value":"0271-9142","type":"print"},{"value":"1573-2592","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,2,20]]},"assertion":[{"value":"7 May 2024","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"9 February 2025","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"20 February 2025","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The authors declare no competing interests.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing Interests"}}],"article-number":"77"}}