{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,6]],"date-time":"2026-09-06T21:49:25Z","timestamp":1788731365455,"version":"build-2803163510"},"reference-count":42,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2024,1,2]],"date-time":"2024-01-02T00:00:00Z","timestamp":1704153600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2024,1,2]],"date-time":"2024-01-02T00:00:00Z","timestamp":1704153600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100020274","name":"EUSA Pharma","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100020274","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["R01HL141408"],"award-info":[{"award-number":["R01HL141408"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000038","name":"U.S. Food and Drug Administration","doi-asserted-by":"publisher","award":["R01FD007632"],"award-info":[{"award-number":["R01FD007632"]}],"id":[{"id":"10.13039\/100000038","id-type":"DOI","asserted-by":"publisher"}]},{"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":["Br J Haematol"],"published-print":{"date-parts":[[2024,3]]},"abstract":"<jats:title>Summary<\/jats:title>\n                  <jats:p>Idiopathic multicentric Castleman disease (iMCD) is a rare haematological disorder characterized by generalized lymphadenopathy with atypical histopathological features and systemic inflammation caused by a cytokine storm involving interleukin\u20106 (IL\u20106). Three clinical subtypes are recognized: thrombocytopenia, anasarca, fever, renal dysfunction, organomegaly (iMCD\u2010TAFRO); idiopathic plasmacytic lymphadenopathy (iMCD\u2010IPL), involving thrombocytosis and hypergammaglobulinaemia; and iMCD\u2010not otherwise specified (iMCD\u2010NOS), which includes patients who do not meet criteria for the other subtypes. Disease pathogenesis is poorly understood, with potential involvement of infectious, clonal and\/or autoimmune mechanisms. To better characterize iMCD clinicopathology and gain mechanistic insights into iMCD, we analysed complete blood counts, other clinical laboratory values and blood smear morphology among 63 iMCD patients grouped by clinical subtype. Patients with iMCD\u2010TAFRO had large platelets, clinical severity associated with lower platelet counts and transfusion\u2010resistant thrombocytopenia, similar to what is observed with immune\u2010mediated destruction of platelets in immune thrombocytopenic purpura. Conversely, elevated platelet counts in iMCD\u2010IPL were associated with elevated IL\u20106 and declined following anti\u2010IL\u20106 therapy. Our data suggest that autoimmune mechanisms contribute to the thrombocytopenia in at least a portion of iMCD\u2010TAFRO patients whereas IL\u20106 drives thrombocytosis in iMCD\u2010IPL, and these mechanisms likely contribute to disease pathogenesis.<\/jats:p>","DOI":"10.1111\/bjh.19279","type":"journal-article","created":{"date-parts":[[2024,1,2]],"date-time":"2024-01-02T18:53:56Z","timestamp":1704221636000},"page":"921-930","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Immune\u2010mediated thrombocytopenia and IL\u20106\u2010mediated thrombocytosis observed in idiopathic multicentric Castleman disease"],"prefix":"10.1111","volume":"204","author":[{"given":"Ayelet I.","family":"Rubenstein","sequence":"first","affiliation":[{"name":"Center for Cytokine Storm Treatment &amp; Laboratory, Department of Medicine, Perelman School of Medicine University of Pennsylvania  Philadelphia Pennsylvania USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sheila K.","family":"Pierson","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":[{"vocabulary":"crossref","role":"author"}]},{"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":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mateo Sarmiento","family":"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":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael V.","family":"Gonzalez","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":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ira D.","family":"Milller","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":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joshua D.","family":"Brandstadter","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":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2145-8347","authenticated-orcid":false,"given":"Melanie D.","family":"Mumau","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":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7367-8184","authenticated-orcid":false,"given":"David C.","family":"Fajgenbaum","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":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,1,2]]},"reference":[{"key":"e_1_2_14_2_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2016-10-746933"},{"key":"e_1_2_14_3_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2018-05-848671"},{"key":"e_1_2_14_4_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2019000931"},{"key":"e_1_2_14_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.23291"},{"key":"e_1_2_14_6_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V96.6.2069.h8002069_2069_2073"},{"key":"e_1_2_14_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.24242"},{"key":"e_1_2_14_8_1","doi-asserted-by":"publisher","DOI":"10.3960\/jslrt.53.87"},{"issue":"10","key":"e_1_2_14_9_1","first-page":"2029","article-title":"Proposed diagnostic criteria, disease severity classification, and treatment strategy for a novel disorder; TAFRO syndrome","volume":"57","author":"Masaki Y","year":"2016","journal-title":"Rinsho Ketsueki"},{"issue":"5","key":"e_1_2_14_10_1","first-page":"320","article-title":"Thrombocytopenia with mild bone marrow fibrosis accompanied by fever, pleural effusion, ascites and hepatosplenomegaly","volume":"51","author":"Takai K","year":"2010","journal-title":"Rinsho Ketsueki"},{"key":"e_1_2_14_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.26292"},{"key":"e_1_2_14_12_1","doi-asserted-by":"publisher","DOI":"10.3960\/jslrt.53.57"},{"key":"e_1_2_14_13_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijms231810301"},{"key":"e_1_2_14_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.25123"},{"key":"e_1_2_14_15_1","article-title":"Longitudinal, natural history study reveals the disease burden of idiopathic multicentric Castleman disease","author":"Bustamante S","year":"2023","journal-title":"Haematologica"},{"key":"e_1_2_14_16_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2003-08-2724"},{"key":"e_1_2_14_17_1","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2020004016"},{"key":"e_1_2_14_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1470-2045(14)70319-5"},{"key":"e_1_2_14_19_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0015004"},{"key":"e_1_2_14_20_1","volume-title":"Clinical methods: the history, physical, and laboratory examinations","author":"Lynch EC","year":"1990"},{"key":"e_1_2_14_21_1","doi-asserted-by":"publisher","DOI":"10.1002\/hon.2969"},{"key":"e_1_2_14_22_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra010501"},{"key":"e_1_2_14_23_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1987.tb02322.x"},{"key":"e_1_2_14_24_1","doi-asserted-by":"publisher","DOI":"10.1097\/00043426-200312001-00013"},{"key":"e_1_2_14_25_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2010-08-302984"},{"key":"e_1_2_14_26_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.86.15.5953"},{"key":"e_1_2_14_27_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V74.5.1545.1545"},{"key":"e_1_2_14_28_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V78.6.1467.1467"},{"issue":"11","key":"e_1_2_14_29_1","first-page":"1095","article-title":"The role of interleukin 6 and interleukin 1 in megakaryocyte development","volume":"17","author":"Warren MK","year":"1989","journal-title":"Exp Hematol"},{"key":"e_1_2_14_30_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2006.06343.x"},{"key":"e_1_2_14_31_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0038-1650032"},{"key":"e_1_2_14_32_1","doi-asserted-by":"publisher","DOI":"10.3390\/jcm10040594"},{"key":"e_1_2_14_33_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.12349"},{"key":"e_1_2_14_34_1","doi-asserted-by":"publisher","DOI":"10.1515\/labmed-2020-0007"},{"key":"e_1_2_14_35_1","doi-asserted-by":"publisher","DOI":"10.1172\/jci.insight.135031"},{"key":"e_1_2_14_36_1","doi-asserted-by":"publisher","DOI":"10.1111\/ejh.13705"},{"key":"e_1_2_14_37_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V85.9.2347.bloodjournal8592347"},{"key":"e_1_2_14_38_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V85.11.3066.bloodjournal85113066"},{"issue":"6","key":"e_1_2_14_39_1","first-page":"935","article-title":"Phase I multicenter trial of interleukin 6 therapy after autologous bone marrow transplantation in advanced breast cancer","volume":"15","author":"Lazarus HM","year":"1995","journal-title":"Bone Marrow Transplant"},{"key":"e_1_2_14_40_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1995.tb05148.x"},{"key":"e_1_2_14_41_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V84.5.1434.1434"},{"key":"e_1_2_14_42_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.1995.13.10.2585"},{"key":"e_1_2_14_43_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.1993.11.3.499"}],"container-title":["British Journal of Haematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/bjh.19279","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/bjh.19279","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/bjh.19279","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,2,16]],"date-time":"2026-02-16T21:20:14Z","timestamp":1771276814000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/bjh.19279"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,1,2]]},"references-count":42,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2024,3]]}},"alternative-id":["10.1111\/bjh.19279"],"URL":"https:\/\/doi.org\/10.1111\/bjh.19279","archive":["Portico"],"relation":{},"ISSN":["0007-1048","1365-2141"],"issn-type":[{"value":"0007-1048","type":"print"},{"value":"1365-2141","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,1,2]]},"assertion":[{"value":"2023-10-02","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-12-19","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-01-02","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}