[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"detail-sidebar-cat-0-en-105":3,"doc-seo-385320-105":59,"doc-detail-385320-en":130},{"code":4,"msg":5,"data":6},0,"success",[7,13,18,23,28,33,38,43,48,51,55],{"id":8,"doc_module":4,"doc_module_name":9,"category_name":10,"show_sort_weight":11,"slug":12},1,"Document","Story & Novel",90,"story-novel",{"id":14,"doc_module":4,"doc_module_name":9,"category_name":15,"show_sort_weight":16,"slug":17},2,"Literature",80,"literature",{"id":19,"doc_module":4,"doc_module_name":9,"category_name":20,"show_sort_weight":21,"slug":22},4,"Exam",70,"exam",{"id":24,"doc_module":4,"doc_module_name":9,"category_name":25,"show_sort_weight":26,"slug":27},5,"Comic",60,"comic",{"id":29,"doc_module":4,"doc_module_name":9,"category_name":30,"show_sort_weight":31,"slug":32},6,"Technology",50,"technology",{"id":34,"doc_module":4,"doc_module_name":9,"category_name":35,"show_sort_weight":36,"slug":37},7,"Healthcare",40,"healthcare",{"id":39,"doc_module":4,"doc_module_name":9,"category_name":40,"show_sort_weight":41,"slug":42},8,"Research & Report",30,"research-report",{"id":44,"doc_module":4,"doc_module_name":9,"category_name":45,"show_sort_weight":46,"slug":47},9,"Religion & Spirituality",20,"religion-spirituality",{"id":46,"doc_module":4,"doc_module_name":9,"category_name":49,"show_sort_weight":46,"slug":50},"World Cup","world-cup",{"id":52,"doc_module":4,"doc_module_name":9,"category_name":53,"show_sort_weight":52,"slug":54},10,"Lifestyle","lifestyle",{"id":56,"doc_module":4,"doc_module_name":9,"category_name":57,"show_sort_weight":24,"slug":58},19,"General","general",{"code":4,"msg":60,"data":61},"ok",{"site_id":62,"language":63,"slug":64,"title":65,"keywords":66,"description":67,"schema_data":68,"social_meta":123,"head_meta":125,"extra_data":127,"updated_unix":129},105,"en","long-term-outcomes-among-adults-with-langerhans-cell-histiocytosis","Long-term outcomes among adults with Langerhans cell histiocytosis","","Long-term clinical outcomes in adults with Langerhans cell histiocytosis (LCH) remain insufficiently characterized. A retrospective record review evaluated 219 adults (≥18 years), describing presentation patterns and survival endpoints at a median follow-up of 74 months. Five-year progression-free survival was 58.3% with median PFS estimated at 83 months, while 5- and 10-year overall survival were 88.7% and 74.5%. Risk-organ involvement and second cancers were linked to worse mortality, with most LCH-related deaths within 5 years. After 5 years, non-LCH causes dominated, supporting survivorship program development.",{"@graph":69,"@context":122},[70,84,105],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":35,"@type":76,"position":81},"https://docshare.wps.com/document/healthcare/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/long-term-outcomes-among-adults-with-langerhans-cell-histiocytosis/385320/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/long-term-outcomes-among-adults-with-langerhans-cell-histiocytosis/385320.png","ImageObject",300,407,{"name":92,"@type":93},"Ben ","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-10-29","2026-09-24",true,{"@type":102,"interactionType":103,"userInteractionCount":52},"InteractionCounter",{"@type":104},"ViewAction",{"@type":106,"mainEntity":107},"FAQPage",[108,114,118],{"name":109,"@type":110,"acceptedAnswer":111},"What study design and cohort were used to evaluate long-term outcomes in adults with LCH?","Question",{"text":112,"@type":113},"The study conducted a retrospective record review of 219 adults (age ≥18 years) with LCH, followed with a median follow-up of 74 months.","Answer",{"name":115,"@type":110,"acceptedAnswer":116},"What survival outcomes were reported for adults with LCH?",{"text":117,"@type":113},"Five-year progression-free survival was 58.3% and median progression-free survival was estimated at 83 months. Median overall survival was not reached, with 5- and 10-year OS rates of 88.7% and 74.5%, respectively.",{"name":119,"@type":110,"acceptedAnswer":120},"Which factors were associated with worse prognosis and mortality?",{"text":121,"@type":113},"Risk organ involvement (liver, spleen, or bone marrow) was associated with worse progression-free survival and overall survival. Risk organ involvement also increased mortality risk, while BRAFV600E was not associated with risk organ involvement or survival.","https://schema.org",{"og:url":83,"og:type":124,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":126,"canonical":83},"index,follow",{"doc_id":128,"site_id":62},385320,1790342444,{"code":4,"msg":5,"data":131},{"doc_id":128,"user_id":132,"nickname":92,"user_avatar":133,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":134,"file_id":135,"file_url":136,"file_type":137,"file_size":138,"view_count":52,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":139,"language":140,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":141,"faqs":142,"seo_title":143,"seo_description":67,"update_tm":144,"read_time":145},2336478951081,"https://ap-avatar.wpscdn.com/davatar_276721f389ce27ea32af1340a28f341c","REGULAR ARTICLE  \nLong-term outcomes among adults with Langerhans cell histiocytosis  \nGaurav Goyal, 1, 2 Aldo A. Acosta-Medina,3 Jithma P. Abeykoon,4 Chen Dai,5 Aishwarya Ravindran,6 Robert Vassallo,7 Jay H. Ryu,7 Mithun V. Shah,4 N. Nora Bennani,4 Jason R. Young,8 Corrie R. Bach,9 Gordon J. Ruan,4 Saurabh Zanwar,4 W. Oliver Tobin, 10 Matthew J. Koster, 11 Caroline J. Davidge-Pitts, 12 Lucinda M. Gruber, 12 Surendra Dasari, 13 Karen L. Rech, 14 and Ronald S. Go,4 on behalf of the Mayo Clinic-University of Alabama at Birmingham Histiocytosis Working Group  \n1 Division of Hematology-Oncology, University of Alabama at Birmingham, Birmingham, AL; 2 Division of Hematology, 3 Department of Internal Medicine, and 4 Division of Hematology, Mayo Clinic, Rochester, MN; 5 Institute for Cancer Outcomes and Survivorship, University of Alabama at Birmingham, Birmingham, AL; 6 Department of Pathology, University of Alabama at Birmingham, Birmingham AL; 7 Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN; 8 Department of Radiology, Mayo Clinic, Jacksonville, FL; and 9 Department of Radiology, 10 Department of Neurology, 11 Division of Rheumatology, 12 Division of Endocrinology, Diabetes, and Nutrition, 13 Division of Biomedical Statistics and Informatics, Department of Quantitative Health Sciences, and 14 Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN  \nKey Points  \n• Adults with LCH have a higher risk of mortality from non-LCH causes such as chronic obstructive pulmonary disease and second primary malignancies.  \n• Most LCH-related deaths occurred within 5 years of diagnosis, with other causes predominating beyond that period.  \nAdvances in the treatment of Langerhans cell histiocytosis (LCH) have resulted in a growing survivor population. There is a lack of data on long-term outcomes among adults with LCH. We conducted a retrospective record review of 219 adults (aged ≥18 years) with LCH. Most common presentation was multisystem (34.2%), followed by single-system pulmonary (32%), unifocal (28.3%), and single-system multifocal (5.5%) LCH. Risk organ involvement (the liver, spleen, or bone marrow) was seen in 8.7% of cases, and 40 of 88 (45.5%) tested cases were BRAFV600E. At a median follow-up of 74 months, 5-year progression-free survival (PFS) was 58 .3% and estimated median PFS was 83 months. Median overall survival (OS) was not reached; 5- and 10-year OS rates were 88.7% and 74.5%, respectively. Risk organ involvement was associated with worse PFS (hazard ratio [HR], 4 .5) and OS (HR, 10 .8) . BRAFV600E was not associated with risk organ involvement or survival. When compared with matched unaffected US population, individuals with LCH had a significantly higher risk of overall mortality (standardized mortality ratio [SMR], 2.66), specifically among those aged \u003C55 years at diagnosis (SMR, 5.94) and those with multisystem disease (SMR, 4.12) . Second cancers occurred in 16.4% cases, including diverse hematologic and solid organ malignancies. LCH-associated deaths constituted 36.1% of deaths and occurred within 5 years of diagnosis. After 5 years, non-LCH causes of death, including second cancers, chronic obstructive pulmonary disease, and cardiovascular diseases, predominated. Our study highlights, to our knowledge, for the first time, that adults with LCH experience early and late mortality from non-LCH causes and the need for development of targeted survivorship programs to improve outcomes.  \nIntroduction  \nLangerhans cell histiocytosis (LCH) is a rare histiocytic disorder, with manifestations ranging from indolent unifocal disease to multisystem disease, causing morbidity and mortality.1 Over the last decade, our understanding of LCH has been revolutionized by the discovery of the clonal  \nSubmitted 11 May 2023; accepted 29 August 2023; prepublished online on Blood Advances First Edition 12 September 2023. [https://doi.org/10.1182/](https://doi.org/10.1182/)[ ](https://doi.org/10.11","cbCaisIgfW7UjcR3","https://ap.wps.com/l/cbCaisIgfW7UjcR3","pdf",624634,11,"English","# Key Points\n# Introduction\n## Background and rationale\n## MAPK–ERK pathway and disease framing\n## Need for adult outcome data","[{\"question\":\"What study design and cohort were used to evaluate long-term outcomes in adults with LCH?\",\"answer\":\"The study conducted a retrospective record review of 219 adults (age ≥18 years) with LCH, followed with a median follow-up of 74 months.\"},{\"question\":\"What survival outcomes were reported for adults with LCH?\",\"answer\":\"Five-year progression-free survival was 58.3% and median progression-free survival was estimated at 83 months. Median overall survival was not reached, with 5- and 10-year OS rates of 88.7% and 74.5%, respectively.\"},{\"question\":\"Which factors were associated with worse prognosis and mortality?\",\"answer\":\"Risk organ involvement (liver, spleen, or bone marrow) was associated with worse progression-free survival and overall survival. Risk organ involvement also increased mortality risk, while BRAFV600E was not associated with risk organ involvement or survival.\"}]","Long-term outcomes among adults with Langerhans cell histiocytosis | PDF",1790267976,28]