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Bone marrow evaluation reveals an increased number of monocytes and mature B cells along with decreased granulocytes. However, little is known about the subpopulations of peripheral blood cells, and few previous publications have been based on a small number of patients. Here, we report a comprehensive immunophenotypic analysis from a cohort of 37 SDS patients who display impairment mostly in the myeloid compartment with a deficiency also in the number of B cells and CD4\/CD8 double\u2010negative T cells.<\/jats:p>","DOI":"10.1002\/pbc.27597","type":"journal-article","created":{"date-parts":[[2019,1,3]],"date-time":"2019-01-03T03:32:14Z","timestamp":1546486334000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Peripheral blood immunophenotyping in a large cohort of patients with Shwachman\u2013Diamond syndrome"],"prefix":"10.1002","volume":"66","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6849-4487","authenticated-orcid":false,"given":"Valentino","family":"Bezzerri","sequence":"first","affiliation":[{"name":"Cystic Fibrosis Center Azienda Ospedaliero Universitaria Ospedali Riuniti  Ancona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio","family":"Vella","sequence":"additional","affiliation":[{"name":"Unit of Immunology Azienda Ospedaliera Universitaria Integrata  Verona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gianfranco Di","family":"Gennaro","sequence":"additional","affiliation":[{"name":"Department of Pathology and Diagnostics Azienda Ospedaliera Universitaria Integrata  Verona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Riccardo","family":"Ortolani","sequence":"additional","affiliation":[{"name":"Unit of Immunology Azienda Ospedaliera Universitaria Integrata  Verona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Nicolis","sequence":"additional","affiliation":[{"name":"Unit of Transfusion Medicine Azienda Ospedaliera Universitaria Integrata  Verona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8698-9547","authenticated-orcid":false,"given":"Simone","family":"Cesaro","sequence":"additional","affiliation":[{"name":"Unit of Pediatric Hematology Oncology Azienda Ospedaliera Universitaria Integrata  Verona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benedetta","family":"Fabrizzi","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Center Azienda Ospedaliero Universitaria Ospedali Riuniti  Ancona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincenzo","family":"Bronte","sequence":"additional","affiliation":[{"name":"Unit of Immunology Azienda Ospedaliera Universitaria Integrata  Verona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seth J.","family":"Corey","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology\/Oncology and Stem Cell Transplantation Cleveland Clinic  Cleveland Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Cipolli","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Center Azienda Ospedaliero Universitaria Ospedali Riuniti  Ancona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,1,2]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng1062"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1101\/gad.623011"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jbior.2017.09.002"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1159\/000055858"},{"key":"e_1_2_7_6_1","first-page":"45","article-title":"Analysis of risk factors for myelodysplasias, leukemias and death from infection among patients with congenital neutropenia. 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