DOI: 10.12890/2024_005040
Prognostic impact of aetiology in adult hemophagocytic lymphohistiocytosis: insights from an intensive care unit experience
Authors
ritanoversadesousa@gmail.com
Keywords
Hemophagocytic lymphohistiocytosis, Epstein-Barr virus, NK-leukemia, organizing pneumonia, viral infectionsAbstract
Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory syndrome marked by excessive immune activation. It can be triggered by various factors, including infections, malignancies, and autoimmune diseases, making the diagnosis challenging due to its overlap with other severe conditions.
Case reports: We discuss two intensive care unit (ICU) cases illustrating the diverse manifestations of HLH and the critical importance of early recognition and treatment. The first case involves natural killer-cell leukaemia, and the second, a suspected viral trigger. Both highlight the necessity of a multidisciplinary approach in diagnosis and management, emphasizing the complexity of HLH in ICU settings.
Conclusions: High mortality rates, particularly in malignancy-associated HLH, underscore the importance of tailored treatment strategies based on the underlying aetiology.
References
- Shakoory B, Geerlinks A, Wilejto M, Kernan K, Hines M, Romano M, et al. The 2022 EULAR/ACR Points to Consider at the Early Stages of Diagnosis and Management of Suspected Haemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome (HLH/MAS). Arthritis Rheumatol 2023;75:1714-1732.
- Scott RB, Robb-Smith AHT. Histiocytic medullary reticulosis. Lancet 1939;234:194–198.
- Hayden A, Park S, Giustini D, Lee AY, Chen LY. Hemophagocytic syndromes (HPSs) including hemophagocytic lymphohistiocytosis (HLH) in adults: A systematic scoping review. Blood Rev 2016;30:411-420.
- Hines MR, von Bahr Greenwood T, Beutel G, Beutel K, Hays JA, Horne A, et al. Consensus-Based Guidelines for the Recognition, Diagnosis, and Management of Hemophagocytic Lymphohistiocytosis in Critically Ill Children and Adults. Crit Care Med 2022;50:860-872.
- Henter JI, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007;48:124-131.
- Fardet L, Galicier L, Lambotte O, Marzac C, Aumont C, Chahwan D, et al. Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. Arthritis Rheumatol 2014;66:2613-2620.
- Jordan MB, Allen CE, Greenberg J, Henry M, Hermiston ML, Kumar A. Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: Recommendations from the North American Consortium for Histiocytosis (NACHO). Pediatr Blood Cancer 2019;66:e27929.
- Ramos-Casals M, Brito-Zerón P, López-Guillermo A, Khamashta MA, Bosch X. Adult haemophagocytic syndrome. Lancet 2014;383:1503-1516.
- Brisse E, Matthys P, Wouters CH. Understanding the spectrum of haemophagocytic lymphohistiocytosis: update on diagnostic challenges and therapeutic options. Br J Haematol 2016;174:175-187.
- Bauchmuller K, Manson JJ, Tattersall R, Brown M, McNamara C, Singer M, et al. Haemophagocytic lymphohistiocytosis in adult critical care. J Intensive Care Soc 2020;21:256-268.
- Knaak C, Nyvlt P, Schuster FS, Spies C, Heeren P, Schenk T, et al. Hemophagocytic lymphohistiocytosis in critically ill patients: diagnostic reliability of HLH-2004 criteria and HScore. Crit Care 2020;24:244.
- Gomes R, Padrão E, Dabó H, Soares Pires F, Mota P, Melo N, et al. Acute fibrinous and organizing pneumonia: A report of 13 cases in a tertiary university hospital. Medicine (Baltimore) 2016;95:e4073.
- Liu L, Bashir H, Awada H, Alzubi J, Lane J. Hemophagocytic Lymphohistiocytosis Complicated by Acute Respiratory Distress Syndrome and Multiorgan Failure. J Investig Med High Impact Case Rep 2021;9:23247096211052180.
