{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,30]],"date-time":"2026-01-30T08:55:38Z","timestamp":1769763338134,"version":"3.49.0"},"reference-count":30,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2024,8,29]],"date-time":"2024-08-29T00:00:00Z","timestamp":1724889600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100017559","name":"Recordati Rare Diseases","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100017559","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["European J of Haematology"],"published-print":{"date-parts":[[2024,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Idiopathic multicentric Castleman disease (iMCD) is a rare inflammatory disorder mediated by excessive proinflammatory cytokine signaling, most notably by interleukin 6 (IL\u20106). IL\u20106\u2010induced extramedullary hematopoiesis (EMH) has been reported in murine models of iMCD. Herein we present four cases of iMCD with EMH in humans.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case Series<\/jats:title><jats:p>The index case is a 24\u2010year\u2010old white woman who presented with pancytopenia, hepatosplenomegaly, and diffuse lymphadenopathy (LAD) with EMH in core lymph node biopsies. We then searched ACCELERATE, a Castleman disease (CD) natural history registry, and identified three additional CD cases with EMH reported in biopsies: A 23\u2010year\u2010old Asian man with fatigue, edema, LAD, and splenomegaly; a 20\u2010year\u2010old white man with fever, dyspnea, LAD, and hepatosplenomegaly; and a 50\u2010year\u2010old white man with constitutional symptoms, LAD, and myelodysplastic syndrome in bone marrow with a KRAS mutation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>All four patients presented with thrombocytopenia and fever and\/or markedly elevated C\u2010reactive protein. Patient 1 had iMCD\u2010NOS (not otherwise specified) with severe thrombocytopenia, reticulin fibrosis in bone marrow, small volume LAD and organomegaly but no anasarca. The other three patients had iMCD\u2010TAFRO (thrombocytopenia, anasarca, reticulin fibrosis, renal dysfunction, organomegaly). Two had mixed CD and two had hypervascular CD in lymph nodes. All four had bone marrow hypercellularity and megakaryocyte hyperplasia and two had reticulin fibrosis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>This case series demonstrates that EMH can be seen in CD, particularly in iMCD\u2010TAFRO. Given the similarity of this finding to previous murine models of IL\u20106\u2010induced marrow and lymph node changes we hypothesize that this is an IL\u20106\u2010mediated phenomenon.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ejh.14295","type":"journal-article","created":{"date-parts":[[2024,8,30]],"date-time":"2024-08-30T05:09:56Z","timestamp":1724994596000},"page":"833-841","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Idiopathic multicentric Castleman disease with marrow fibrosis and extramedullary hematopoiesis"],"prefix":"10.1111","volume":"113","author":[{"given":"Marley","family":"Blommers","sequence":"first","affiliation":[{"name":"Faculty of Medicine Dalhousie University  Halifax Nova Scotia Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sorin","family":"Selegean","sequence":"additional","affiliation":[{"name":"Department of Pathology Dalhousie University"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Richard K.","family":"Wood","sequence":"additional","affiliation":[{"name":"Department of Pathology Dalhousie University"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mateo","family":"Sarmiento Bustamante","sequence":"additional","affiliation":[{"name":"Center for Cytokine Storm Treatment &amp; Laboratory, Department of Medicine Perelman School of Medicine, University of Pennsylvania  Philadelphia Pennsylvania USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Saishravan","family":"Shyamsundar","sequence":"additional","affiliation":[{"name":"Center for Cytokine Storm Treatment &amp; Laboratory, Department of Medicine Perelman School of Medicine, University of Pennsylvania  Philadelphia Pennsylvania USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E. 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