{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,16]],"date-time":"2026-05-16T06:28:15Z","timestamp":1778912895565,"version":"3.51.4"},"reference-count":20,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2021,10,8]],"date-time":"2021-10-08T00:00:00Z","timestamp":1633651200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Langerhans cell histiocytosis (LCH) is a histiocytic neoplasm that can involve the lungs as single system (LCH\u2010SSL) or multisystem disease (LCH\u2010MSL). The role of full\u2010body radiographic staging to determine whether patients have LCH\u2010SSL or LCH\u2010MSL is unclear. Long\u2010term outcomes of LCH\u2010SSL versus LCH\u2010MSL and multisystem without lung involvement (LCH\u2010MSNL) are unknown. A retrospective study of adult LCH patients seen at our center from January 2000 to 2020 was performed. In Part 1, we addressed utility of whole\u2010body staging imaging among those presenting with isolated pulmonary signs or symptoms. Staging was defined as fluorodeoxyglucose positron emission tomography\u2010computed tomography (CT) or whole\u2010body CT obtained within 3\u2009months of diagnosis. In Part 2, we examined the frequency of developing extra\u2010pulmonary disease over time and mortality in patients with LCH\u2010SSL. In Part 3, we compared the overall survival of LCH\u2010SSL, LCH\u2010MSL, and LCH\u2010MSNL. Part 1: 240 patients with LCH were identified. A total of 112 (47%) had pulmonary signs or symptoms at presentation. Thirty\u2010four (30%) underwent radiographic staging and only one showed evidence of extra\u2010pulmonary disease. Part 2: 108 (45%) were LCH\u2010SSL. Median follow\u2010up duration of 4.5\u2009years (95% confidence interval [CI]: 2.9\u20136.0). None developed extra\u2010pulmonary disease. Part 3: 5\u2010year survival: 94% (95% CI: 84%\u201398%) for LCH\u2010SSL, 78% (95% CI: 59%\u201390%) for LCH\u2010MSL, and 75% (95% CI: 53%\u201389%) for LCH\u2010MSNL. LCH patients presenting with isolated pulmonary signs or symptoms rarely have extra\u2010pulmonary involvement at the time of diagnosis and generally do not develop extra\u2010pulmonary progression. LCH\u2010SSL has the best overall survival, while LCH\u2010MSL and LCH\u2010MSNL have similar clinical outcomes.<\/jats:p>","DOI":"10.1002\/ajh.26355","type":"journal-article","created":{"date-parts":[[2021,9,23]],"date-time":"2021-09-23T06:48:09Z","timestamp":1632379689000},"page":"1604-1610","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["Langerhans cell histiocytosis with lung involvement in isolation and multisystem disease: Staging, natural history, and comparative survival"],"prefix":"10.1002","volume":"96","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2938-3730","authenticated-orcid":false,"given":"Antonious Z.","family":"Hazim","sequence":"first","affiliation":[{"name":"Department of Internal Medicine Mayo Clinic  Rochester Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6969-9169","authenticated-orcid":false,"given":"Gordon J.","family":"Ruan","sequence":"additional","affiliation":[{"name":"Division of Hematology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9847-7207","authenticated-orcid":false,"given":"Marie","family":"Hu","sequence":"additional","affiliation":[{"name":"Division of Hematology\u2010Oncology University of Minnesota  Minneapolis Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4503-440X","authenticated-orcid":false,"given":"Aishwarya","family":"Ravindran","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Karen L.","family":"Rech","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jason R.","family":"Young","sequence":"additional","affiliation":[{"name":"Department of Radiology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christian W.","family":"Cox","sequence":"additional","affiliation":[{"name":"Department of Radiology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jithma P.","family":"Abeykoon","sequence":"additional","affiliation":[{"name":"Division of Hematology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7248-2219","authenticated-orcid":false,"given":"Caleb","family":"Scheckel","sequence":"additional","affiliation":[{"name":"Division of Hematology Mayo Clinic  Rochester Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert","family":"Vassallo","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine Mayo Clinic  Rochester Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9576-2272","authenticated-orcid":false,"given":"Jay H.","family":"Ryu","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine Mayo Clinic  Rochester Minnesota USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"W. 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