<?xml version="1.0" encoding="UTF-8"?> <!DOCTYPE article PUBLIC "-//NLM//DTD JATS (Z39.96) Journal Publishing DTD v1.2d1 20170631//EN" "JATS-journalpublishing1.dtd"> <ArticleSet> <Article> <Journal> <PublisherName>jmedicalcasereports</PublisherName> <JournalTitle>Frontiers in Medical Case Reports</JournalTitle> <PISSN>I</PISSN> <EISSN>S</EISSN> <Volume-Issue>Volume 1; Issue 1, (Jan-Jun,2020)</Volume-Issue> <PartNumber/> <IssueTopic>Multidisciplinary</IssueTopic> <IssueLanguage>English</IssueLanguage> <Season>Cancer Case Reports</Season> <SpecialIssue>N</SpecialIssue> <SupplementaryIssue>N</SupplementaryIssue> <IssueOA>Y</IssueOA> <PubDate> <Year>-0001</Year> <Month>11</Month> <Day>30</Day> </PubDate> <ArticleType>Cancer Case Reports</ArticleType> <ArticleTitle>A Rare Case of Hemophagocytic Lymphohistiocytosis with Concurrent Extra-Pulmonary Sarcoidosis Presenting as Fever of Unknown Origin</ArticleTitle> <SubTitle/> <ArticleLanguage>English</ArticleLanguage> <ArticleOA>Y</ArticleOA> <FirstPage>1</FirstPage> <LastPage>5</LastPage> <AuthorList> <Author> <FirstName>Dharti</FirstName> <LastName>Patel</LastName> <AuthorLanguage>English</AuthorLanguage> <Affiliation/> <CorrespondingAuthor>N</CorrespondingAuthor> <ORCID/> <FirstName>Kinza</FirstName> <LastName>Muzzaffar</LastName> <AuthorLanguage>English</AuthorLanguage> <Affiliation/> <CorrespondingAuthor>Y</CorrespondingAuthor> <ORCID/> <FirstName>Ateeq</FirstName> <LastName>Mubarik</LastName> <AuthorLanguage>English</AuthorLanguage> <Affiliation/> <CorrespondingAuthor>Y</CorrespondingAuthor> <ORCID/> <FirstName>Nirali</FirstName> <LastName>Vassa</LastName> <AuthorLanguage>English</AuthorLanguage> <Affiliation/> <CorrespondingAuthor>Y</CorrespondingAuthor> <ORCID/> <FirstName>Salman</FirstName> <LastName>Muddassir</LastName> <AuthorLanguage>English</AuthorLanguage> <Affiliation/> <CorrespondingAuthor>Y</CorrespondingAuthor> <ORCID/> </Author> </AuthorList> <DOI/> <Abstract>Hemophagocytic Lymphohistiocytosis (HLH) is a rare syndrome with uncontrolled inflammation due to an ineffective immune response to antigens, leading to multiorgan failure if not treated aggressively (Atteritano et al., 2012). HLH can be genetic or acquired. Primary HLH, which is diagnosed at an early age, is associated with immunodeficiency syndromes (Rosado and Kim, 2013).The acquired form, which can occur at any age, has various etiologies including infections, malignancies or rheumatological diseases (Schram and Berliner, 2015; Sawhney et al., 2001). The Histiocyte Society defines the diagnostic criteria for HLH on the basis of few characteristics including fever, cytopenia, splenomegaly, hyperferritinemia, hypertriglyceridemia, decreased fibrinogen, hemophagocytosis, increased IL-2 receptor alpha and decreased natural killer (NK) function. Sarcoidosis is a chronic granulomatous and multisystem inflammatory disease, which is rarely associated with HLH (Sawhney et al., 2001). The rheumatologic form of secondary HLH is also known as macrophage activation syndrome (MAS). The most common rheumatic diseases associated with HLH are juvenile arthritis and systemic lupus erythematosus (SLE). The clinical course of the disease is very aggressive, with the mortality rates ranging from 8% to 22%, therefore early diagnosis is key to initiation of prompt treatment (Abughanimeh et al., 2018). In order to add our contribution to the available literature on this under recognized association, we report a unique case of a 70 year old female who presented with fever of unknown origin and ultimately was diagnosed with sarcoidosis complicated by HLH during her hospital course.</Abstract> <AbstractLanguage>English</AbstractLanguage> <Keywords>Hemophagocytic Lymphohistiocytosis,Macrophage Activation,Cytokine Storm,Immune Dysregulation,Phagocytosis</Keywords> <URLs> <Abstract>https://jmedicalcasereports.org/ubijournal-v1copy/journals/abstract.php?article_id=15131&title=A Rare Case of Hemophagocytic Lymphohistiocytosis with Concurrent Extra-Pulmonary Sarcoidosis Presenting as Fever of Unknown Origin</Abstract> </URLs> <References> <ReferencesarticleTitle>References</ReferencesarticleTitle> <ReferencesfirstPage>16</ReferencesfirstPage> <ReferenceslastPage>19</ReferenceslastPage> <References>Abughanimeh O, Qasrawi A, Ghanimeh MA. Hemophagocytic Lymphohistiocytosis Complicating Systemic Sarcoidosis. Cureus 2018; 10: e2838. Atteritano M, David A, Bagnato G, Beninati C, Frisina A, Iaria C, Bagnato G, Cascio A. Haemophagocytic syndrome in rheumatic patients. A systematic review. Eur Rev Med Pharmacol Sci 2012; 16: 1414-1424. Hadchouel M, Prieur AM, Griscelli C. Acute hemorrhagic, hepatic, and neurologic manifestations in juvenile rheumatoid arthritis: possible relationship to drugs or infection. The Journal of pediatrics 1985; 106: 561-566. Henter JI, Horne A, Aricand;oacute; M, Egeler RM, Filipovich AH, Imashuku S, Ladisch S, McClain K, Webb D, Winiarski J, Janka G. HLH?2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatric blood and; cancer 2007; 48: 124-131. Jordan MB, Allen CE, Weitzman S, Filipovich AH, McClain KL. How I treat hemophagocytic lymphohistiocytosis. Blood 2011; 118: 4041-4052. Kapoor R, Giri PP, Das J. Fulminant Familial Hemophagocytic Lymphohistiocytosis (FHL) in an Infant Masquerading Septic Shock-A case report. Journal of Pediatric Critical Care 2016: 152. Kleynberg RL and Schiller GJ. Secondary hemophagocytic lymphohistiocytosis in adults: an update on diagnosis and therapy. Clin Adv Hematol Oncol 2012; 10: 726-732. Rosado FG and Kim AS. Hemophagocytic LymphohistiocytosisAn Update on Diagnosis and Pathogenesis. American Journal of Clinical Pathology 2013; 139: 713-727. Sawhney S, Woo P, Murray KJ. Macrophage activation syndrome: a potentially fatal complication of rheumatic disorders. Archives of disease in childhood 2001; 85: 421-426. Schram AM and Berliner N. How I treat hemophagocytic lymphohistiocytosis in the adult patient. Blood 2015; 125: 2908-2914.</References> </References> </Journal> </Article> </ArticleSet>