Clinical Hemophagocytic Lymphohistiocytosis (HLH): A Case Series


Authors : Dr. Rishika R Reddy; Dr. Naveen Angadi; Dr. Rohan Bhise

Volume/Issue : Volume 10 - 2025, Issue 3 - March


Google Scholar : https://tinyurl.com/ys4uamt2

Scribd : https://tinyurl.com/mu72y5b5

DOI : https://doi.org/10.38124/ijisrt/25mar249

Google Scholar

Note : A published paper may take 4-5 working days from the publication date to appear in PlumX Metrics, Semantic Scholar, and ResearchGate.

Note : Google Scholar may take 15 to 20 days to display the article.


Abstract : Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening hyperinflammatory syndrome characterized by excessive immune activation. Despite being historically associated with pediatric populations, HLH is increasingly recognized in adults, albeit with diagnostic and therapeutic challenges. This case series evaluates seven adult patients diagnosed with HLH, exploring their clinical presentation, diagnostic challenges, and treatment outcomes. Our findings highlight the need for improved awareness, timely diagnosis, and a tailored approach to managing adult HLH.

References :

  1. Ramos-Casals M, Brito-Zerón P, López-Guillermo A, Khamashta MA, Bosch X. Adult haemophagocytic syndrome. Lancet. 2014 Apr 26;383(9927):1503-1516. doi: 10.1016/S0140-6736(13)61048-X. Epub 2013 Nov 27. Erratum in: Lancet. 2014 Apr 26;383(9927):1464. PMID: 24290661.
  2. 2.Ravelli A, Minoia F, Davì S, Horne A, Bovis F, Pistorio A, Aricò M, Avcin T, Behrens EM, De Benedetti F, Filipovic A, Grom AA, Henter JI, Ilowite NT, Jordan MB, Khubchandani R, Kitoh T, Lehmberg K, Lovell DJ, Miettunen P, Nichols KE, Ozen S, Pachlopnik Schmid J, Ramanan AV, Russo R, Schneider R, Sterba G, Uziel Y, Wallace C, Wouters C, Wulffraat N, Demirkaya E, Brunner HI, Martini A, Ruperto N, Cron RQ. Expert consensus on dynamics of laboratory tests for diagnosis of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. RMD Open. 2016 Jan 19;2(1):e000161. doi: 10.1136/ rmdopen-2015-000161. PMID: 26848401; PMCID: PMC4731834.
  3. 3.Emmenegger U, Schaer DJ, Larroche C, Neftel KA. Haemophagocytic syndromes in adults: current concepts and challenges ahead. Swiss Med Wkly. 2005 May 28;135(21-22):299-314. doi: 10.4414/smw.2005.10976. PMID: 16034684.
  4. 4.Jordan MB, Allen CE, Greenberg J, Henry M, Hermiston ML, Kumar A, Hines M, Eckstein O, Ladisch S, Nichols KE, Rodriguez-Galindo C, Wistinghausen B, McClain KL. Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: Recommendations from the North American Consortium for Histiocytosis (NACHO). Pediatr Blood Cancer. 2019 Nov;66(11):e27929. doi: 10.1002/pbc.27929. Epub 2019 Jul 24. PMID: 31339233; PMCID: PMC7340087.

Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening hyperinflammatory syndrome characterized by excessive immune activation. Despite being historically associated with pediatric populations, HLH is increasingly recognized in adults, albeit with diagnostic and therapeutic challenges. This case series evaluates seven adult patients diagnosed with HLH, exploring their clinical presentation, diagnostic challenges, and treatment outcomes. Our findings highlight the need for improved awareness, timely diagnosis, and a tailored approach to managing adult HLH.

Never miss an update from Papermashup

Get notified about the latest tutorials and downloads.

Subscribe by Email

Get alerts directly into your inbox after each post and stay updated.
Subscribe
OR

Subscribe by RSS

Add our RSS to your feedreader to get regular updates from us.
Subscribe