{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,7,28]],"date-time":"2024-07-28T13:16:15Z","timestamp":1722172575423},"reference-count":25,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2015,2,10]],"date-time":"2015-02-10T00:00:00Z","timestamp":1423526400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Associazione Italiana Sindrome di Shwachman-Diamond (AISS)"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["European J of Haematology"],"published-print":{"date-parts":[[2015,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objectives<\/jats:title><jats:p>Shwachman\u2013Diamond syndrome is a rare disorder characterized by exocrine pancreatic insufficiency, skeletal abnormalities, and bone marrow failure, with high risk of leukemic evolution. The aim of the study was the immunophenotypic characterization of bone marrow cells from patients with Shwachman\u2013Diamond syndrome to assess the maturation pathway of blood progenitor cells and to identify the presence of recurrent abnormalities.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Bone marrow samples from nineteen patients and eleven controls were analyzed by multiparameter flow cytometry.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We found a low frequency of <jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>34+ cells (<jats:italic>P\u00a0<\/jats:italic>=\u00a00.0179) and myeloid progenitors (<jats:italic>P\u00a0<\/jats:italic>=<jats:italic>\u00a0<\/jats:italic>0.025), in the bone marrow of patients with Shwachman\u2013Diamond syndrome as compared to the controls. A significant reduction in the percentage of granulocytes (<jats:italic>P\u00a0<\/jats:italic>=<jats:italic>\u00a0<\/jats:italic>0.002) and an increase of monocytes (<jats:italic>P\u00a0<\/jats:italic>&lt;<jats:italic>\u00a0<\/jats:italic>0.001) were also evident in the bone marrow of patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>On the basis of these observations, future prospective assessments may be useful to verify the contribution of bone marrow immunophenotype in the early identification of the evolution toward aplasia or myelodysplasia.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ejh.12490","type":"journal-article","created":{"date-parts":[[2014,11,17]],"date-time":"2014-11-17T13:59:52Z","timestamp":1416232792000},"page":"308-315","source":"Crossref","is-referenced-by-count":14,"title":["Immunophenotypic analysis of hematopoiesis in patients suffering from Shwachman\u2013Bodian\u2013Diamond Syndrome"],"prefix":"10.1111","volume":"95","author":[{"given":"Angela","family":"Mercuri","sequence":"first","affiliation":[{"name":"Pediatric Hematology University of Verona Verona Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elisa","family":"Cannata","sequence":"additional","affiliation":[{"name":"Pediatric Hematology University of Verona Verona Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Omar","family":"Perbellini","sequence":"additional","affiliation":[{"name":"Section of Hematology Department of Medicine University of Verona Verona Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chiara","family":"Cugno","sequence":"additional","affiliation":[{"name":"Pediatric Hematology University of Verona Verona Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rita","family":"Balter","sequence":"additional","affiliation":[{"name":"Pediatric Hematology University of Verona Verona 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Medicine University of Verona Verona Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marco","family":"Cipolli","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Center University of Verona Verona Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Simone","family":"Cesaro","sequence":"additional","affiliation":[{"name":"Pediatric Hematology University of Verona Verona 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