[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"detail-sidebar-cat-0-en-105":3,"doc-seo-461717-105":59,"doc-detail-461717-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","presentation-diagnosis-and-acute-treatment-of-secondary-hemophagocytic-lymphohistiocytosis-a-case-report","Presentation, Diagnosis, and Acute Treatment of Secondary Hemophagocytic Lymphohistiocytosis - A Case Report","","Secondary hemophagocytic lymphohistiocytosis (HLH) is a rare, immunologically driven disorder with high mortality. It is generally diagnosed using clinical and laboratory criteria. The article presents a case of a 53-year-old woman with multiple prior admissions for fever of unknown origin and fatigue, whose evaluation ultimately led to HLH diagnosis. It reviews the presentation, diagnostic criteria, and acute treatment approaches for secondary HLH to inform future workup and management.",{"@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/presentation-diagnosis-and-acute-treatment-of-secondary-hemophagocytic-lymphohistiocytosis-a-case-report/461717/",{"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/presentation-diagnosis-and-acute-treatment-of-secondary-hemophagocytic-lymphohistiocytosis-a-case-report/461717.png","ImageObject",300,407,{"name":92,"@type":93},"dawugda","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-11-07","2026-09-30",true,{"@type":102,"interactionType":103,"userInteractionCount":14},"InteractionCounter",{"@type":104},"ViewAction",{"@type":106,"mainEntity":107},"FAQPage",[108,114,118],{"name":109,"@type":110,"acceptedAnswer":111},"What is secondary hemophagocytic lymphohistiocytosis (HLH) and why is it dangerous?","Question",{"text":112,"@type":113},"Secondary HLH is an immunologically driven disorder with a high mortality rate. It results from excessively active immune responses that can trigger a hyperinflammatory state, cytopenias, severe infection, sepsis, and organ damage.","Answer",{"name":115,"@type":110,"acceptedAnswer":116},"How is HLH typically diagnosed in this case report?",{"text":117,"@type":113},"Diagnosis is based on clinical and laboratory criteria, using the HLH-2004 framework with updated 2024 criteria. A diagnosis can be made through molecular diagnosis consistent with HLH or by meeting five of seven established criteria.",{"name":119,"@type":110,"acceptedAnswer":120},"What treatments are discussed for secondary HLH?",{"text":121,"@type":113},"Treatment can include chemotherapy such as dexamethasone and etoposide, along with immune therapy and, in some cases, hematopoietic stem cell transplant.","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},461717,1790762234,{"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":14,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":44,"language":139,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":140,"faqs":141,"seo_title":142,"seo_description":67,"update_tm":129,"read_time":143,"preview_page_count":4},3985747870947,"https://ap-avatar.wpscdn.com/davatar_3d24733baf745e90a7e4bdd5f77d97b2","Review began 09/17/2025  \nReview ended 12/02/2025  \nPublished 12/10/2025  \n© Copyright 2025  \nBartholomew et al. This is an open access article distributed under the terms of the Creative Commons Attribution License CCBY 4.0. , which permits unrestricted use , distribution , and reproduction in any medium , provided the original author and source are credited.  \nDOI: 10.7759/cureus.98913  \nOpen Access Case Report  \nPresentation, Diagnosis, and Acute Treatment of Secondary Hemophagocytic Lymphohistiocytosis: A Case Report  \nAlexandra Bartholomew 1, Michael Connick 1, Catherine Loehr 1 , Shane Sanne 1  \n1. Department of Internal Medicine, Louisiana State University Health Sciences Center, New Orleans, USA Corresponding author: Alexandra Bartholomew, [abart3@lsuhsc.edu](abart3@lsuhsc.edu)  \nAbstract  \nSecondary hemophagocytic lymphohistiocytosis (HLH) is a rare, immunologically driven disorder with a high mortality rate. It is typically diagnosed on the basis of clinical and laboratory criteria. We present the case of a 53-year-old woman with multiple previous hospitalizations for fever of unknown origin and fatigue. Her workup ultimately led to a diagnosis of HLH. We discuss the presentation, diagnostic criteria, and clinical treatment of secondary HLH to guide workup and management for future patients affected by this rare disease.  \nCategories: Hematology, Internal Medicine, Allergy/Immunology  \nKeywords: fever of unknown origin, hemophagocytic lymphohistiocytosis, hlh-2004 protocol, hyperinflammatory state, immune system dysregulation, pancytopenia, secondary hemophagocytic lymphohistiocytosis (hlh)  \nIntroduction  \nHemophagocytic lymphohistiocytosis (HLH) is a potentially fatal condition linked to an excessively active immune system. The pathophysiology of both the primary (familial) and secondary (acquired) forms of HLHis commonly attributed to immunologic dysregulation, which can result in overactivity of the cell-mediated immune system [1] . The resulting hyperinflammatory state can lead to cytopenias and ultimately to severe infection, sepsis, end-organ damage, and not infrequently death.  \nThe primary form of HLH largely affects children and is associated with several genetic mutations [2] . The secondary form of HLH has been associated with infection, autoimmunity, immune suppression, organ transplant, and malignancy; while the disease process of this potentially fatal illness is not completely understood, its pathophysiology is thought to be multifactorial [1-3]. Moreover, HLH in adults previously has been estimated to account for less than 1% of admissions to tertiary care centers, though the scarcity of data makes incidence difficult to assess accurately [3] . Diagnostic challenges of HLH derive largely from its widely variable and non-specific presentation. Delays in diagnosis and treatment may further exacerbate the effects of HLH, resulting in deterioration of clinical status. This medical instability may make certain treatment options, such as chemotherapy and stem cell transplant, more difficult to pursue.  \nThe most widely recognized set of diagnostic criteria for both primary and secondary HLH is based upon the HLH-2004 clinical trial [4] . These criteria were recently updated in 2024. Based on the 2024 criteria, a diagnosis of HLH is made through a molecular diagnosis consistent with HLH or by meeting five out of seven established criteria [5] . The HLH-2024 criteria remain the preferred diagnostic guidelines  \ntoday. Additionally, an adult individual 's risk of having HLH can be estimated using the HScore, which consists of a set of weighted clinical and laboratory criteria such as temperature, number of cytopenias, and evidence of hemophagocytosis features on bone marrow aspirate. This tool is recognized to be highly sensitive but less specific.  \nTreatment for secondary HLH can include chemotherapy (such as dexamethasone and etoposide) as well as immune therapy or even hematopoietic stem cell transplant [","cbCaipWtLhsmBmPK","https://ap.wps.com/l/cbCaipWtLhsmBmPK","pdf",2824603,"English","# Abstract\n# Introduction\n# Case Presentation\n## Patient history and presentation\n## Vital signs and laboratory findings","[{\"question\":\"What is secondary hemophagocytic lymphohistiocytosis (HLH) and why is it dangerous?\",\"answer\":\"Secondary HLH is an immunologically driven disorder with a high mortality rate. It results from excessively active immune responses that can trigger a hyperinflammatory state, cytopenias, severe infection, sepsis, and organ damage.\"},{\"question\":\"How is HLH typically diagnosed in this case report?\",\"answer\":\"Diagnosis is based on clinical and laboratory criteria, using the HLH-2004 framework with updated 2024 criteria. A diagnosis can be made through molecular diagnosis consistent with HLH or by meeting five of seven established criteria.\"},{\"question\":\"What treatments are discussed for secondary HLH?\",\"answer\":\"Treatment can include chemotherapy such as dexamethasone and etoposide, along with immune therapy and, in some cases, hematopoietic stem cell transplant.\"}]","Presentation, Diagnosis, and Acute Treatment of Secondary Hemophagocytic Lymphohistiocytosis - A Case Report | PDF",23]