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In fact, the spleen of patients with MF contains malignant hematopoietic stem cells retaining a complete differentiation program, suggesting both a pivotal role of the spleen in maintaining the disease and a tight regulation of hematopoiesis by the splenic microenvironment, in particular by mesenchymal stromal cells (MSCs). Little is known about splenic MSCs (Sp\u2010MSCs), both in normal and in pathological context. In this work, we have in vitro expanded and characterized Sp\u2010MSCs from 25 patients with MF and 13 healthy subjects (HS). They shared similar phenotype, growth kinetics, and differentiation capacity. However, MF Sp\u2010MSCs expressed significant lower levels of nestin, and favored megakaryocyte (Mk) differentiation in vitro at a larger extent than their normal counterpart. Moreover, they showed a significant upregulation of matrix metalloprotease 2 (<jats:italic>MMP2<\/jats:italic>) and fibronectin 1 (<jats:italic>FN1<\/jats:italic>) genes both at mRNA expression and at protein level, and, finally, developed genetic abnormalities which were never detected in HS\u2010derived Sp\u2010MSCs. Our data point toward the existence of a defective splenic niche in patients with MF that could be responsible of some pathological features of the disease, including the increased trafficking of CD34+ cells and the expansion of the megakaryocytic lineage.<\/jats:p>","DOI":"10.1002\/ajh.25047","type":"journal-article","created":{"date-parts":[[2018,1,23]],"date-time":"2018-01-23T07:36:38Z","timestamp":1516692998000},"page":"615-622","source":"Crossref","is-referenced-by-count":14,"title":["The spleen of patients with myelofibrosis harbors defective mesenchymal stromal cells"],"prefix":"10.1002","volume":"93","author":[{"given":"Maria Antonietta","family":"Avanzini","sequence":"first","affiliation":[{"name":"Pediatric Onco\u2010Hematology\/Cell Factory, IRCCS Policlinico San Matteo Foundation Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vittorio","family":"Abbonante","sequence":"additional","affiliation":[{"name":"Department of Molecular Medicine 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