Video: Every Case Tells a Story| Webinar: ACR/CHEST ILD Guidelines in Practice

An official publication of the ACR and the ARP serving rheumatologists and rheumatology professionals

Diagnosing & Treating Hemophagocytic Lymphohistiocytosis in Adults

Srujana Pachigolla, MD, & Adegbenga Bankole, MD  |  Issue: June 2020  |  

Adegbenga Bankole, MDAdegbenga Bankole, MD, is chief of rheumatology at the Carilion Clinic and is an associate professor of medicine at Virginia Tech Carilion School of Medicine, Roanoke, Va.

References

  1. Madkaikar M, Shabrish S, Mukesh Desai M. Current updates on classification, diagnosis and treatment of hemophagocytic lymphohistiocytosis (HLH). Indian J Pediatr. 2016 May;83(5):434–443.
  2. Hayden A, Park S, Giustini D, et al. Hemophagocytic syndromes (HPSs) including hemophagocytic lymphohistiocytosis (HLH) in adults: A systematic scoping review. Blood Rev. 2016 Nov;30(6):411–420.
  3. Kumar V, Sharma B, Nigam AS. Case series of hemophagocytic lymphohistiocytosis from a tertiary care centre: An underdiagnosed entity. Turk Patoloji Derg. 2019 Apr 12; 35(3):207–212.
  4. Henter JI, Elinder G, Söder O, Ost A. Incidence in Sweden and clinical features of familial hemophagocytic lymphohistiocytosis. Acta Paediatr Scand. 1991 Apr;80(4):428–435.
  5. Henter JI, Horne A, Aricó M, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007 Feb;48(2):124–131.
  6. Meena NK, Sinokrot O, Duggal A, et al. The performance of diagnostic criteria for hemophagocytic lymphohistiocytosis in critically ill patients. J Intensive Care Med. 2019 Mar 12:885066619837139.
  7. Gupta A, Weitzman S, Abdelhaleem M. The role of hemophagocytosis in bone marrow aspirates in the diagnosis of hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2008 Feb;50(2):192–194.
  8. Fardet L, Galicier L, Lambotte O, et al. Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. Arthritis Rheumatol. 2014 Sep;66(9):2613–2620.
  9. Lerkvaleekul B, Vilaiyuk S. Macrophage activation syndrome: early diagnosis is key. Open Access Rheumatol. 2018 Aug 31;10:117–128.
  10. Bracaglia C, Prencipe G, De Benedetti F. Macrophage activation syndrome: Different mechanisms leading to a one clinical syndrome. Pediatr Rheumatol Online J. 2017 Jan 17;15(1):5.
  11. La Rosée P, Horne A, Hines M, et al. Recommendations for the management of hemophagocytic lymphohistiocytosis in adults. Blood. 2019 Jun 6;133(23):2465–2477.

Related Articles
    Fellows' Forum Case Report: Hemophagocytic Lymphohistiocytosis

    Fellows’ Forum Case Report: Hemophagocytic Lymphohistiocytosis

    The patient was a 48-year-old woman who saw her primary care physician for a flu-like illness three months prior to admission. Her symptoms initially improved, but recurred one month later; she was treated symptomatically, and again symptoms resolved. Two months later, she presented to an outside facility’s emergency department with fever to 103ºF, with associated…

    Figure 1A–C: Arrows denote hemophagocytic histiocytes.

    Case Report: Too Many Activated Immune Cells in a 9-Month-Old Boy

    Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease of immune dysregulation characterized by unchecked inflammatory responses leading to end-organ dysfunction. Primary HLH results from inherited mutations that impair the capacity for immune regulation; secondary HLH arises from the inappropriate response to an immune stimulus, such as infection, malignancy or autoimmunity. What is less well known is…

    ANCA-Associated Vasculitis Trial Results May Change Standard of Care

    SNOWMASS VILLAGE, COLO.—The study of anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is ongoing, and research results should help improve treatment for this patient pop­ulation. Key trials and therapeutic options were discussed at the 2020 ACR Winter Symposium during the session, Update on the Treatment of ANCA-Associated Vasculitis, by Peter A. Merkel, MD, MPH, chief of…