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VOLUME 26 , ISSUE 5 ( May, 2022 ) > List of Articles

Original Article

Clinical Profile, Treatment, and Outcome of Patients with Secondary Hemophagocytic Lymphohistiocytosis in Critically Ill Patients: A Prospective Observational Study

Farhan Fazal, DK Mitra, G Satpathy, SK Panda, PK Chaturvedi, Naval K Vikram, RM Pandey, Naveet Wig

Keywords : BM score, Ferritin, Hemophagocytic lymphohistiocytosis, H score, Infection

Citation Information : Fazal F, Mitra D, Satpathy G, Panda S, Chaturvedi P, Vikram NK, Pandey R, Wig N. Clinical Profile, Treatment, and Outcome of Patients with Secondary Hemophagocytic Lymphohistiocytosis in Critically Ill Patients: A Prospective Observational Study. Indian J Crit Care Med 2022; 26 (5):562-565.

DOI: 10.5005/jp-journals-10071-24136

License: CC BY-NC 4.0

Published Online: 30-04-2022

Copyright Statement:  Copyright © 2022; The Author(s).


Introduction: The objective of the study was to evaluate the clinical profile and outcome of patients with secondary hemophagocytic lymphohistiocytosis (HLH) in critically ill patients. Materials and methods: A prospective observational study was conducted where critically ill adult patients presenting with fever and bicytopenia were evaluated according to the HLH-2004 diagnostic criteria for the presence of secondary HLH. The underlying trigger, clinical profile, treatment, and outcome of patients with HLH were analyzed. Results: Of the 76 critically ill patients with fever and bicytopenia, 33 (43%) patients were diagnosed with HLH. The following triggers for HLH were identified: bacterial infections (23%), fungal infections (10%), viral infections (10%), parasitic infections (10%), autoimmune diseases (13%), and malignancy (8%). A total of 78% of the HLH cases received steroids, but the use of steroids was not associated with improvement in mortality. Conclusion: There is a high prevalence of HLH in patients presenting with fever and bicytopenia in critically ill adult patients. Infections were identified as the most common trigger of HLH.

  1. Jordan MB, Allen CE, Weitzman S, Filipovich AH, McClain KL. How I treat hemophagocytic lymphohistiocytosis. Blood 2011;118(15):4041–4052. DOI: 10.1182/blood-2011-03-278127.
  2. Levy L, Nasereddin A, Rav-Acha M, Kedmi M, Rund D, Gatt ME. Prolonged fever, hepatosplenomegaly, and pancytopenia in a 46-year-old woman. PLoS Med 2009;6(4):e1000053. DOI: 10.1371/journal.pmed.1000053.
  3. Henter JI, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatric Blood Cancer 2007;48(2):124–131. DOI: 10.1002/pbc.21039.
  4. Janka GE. Familial and acquired hemophagocytic lymphohistiocytosis. Annu Rev Med 2012;63:233–246. DOI: 10.1146/annurev-med-041610-134208.
  5. Weitzman S. Approach to hemophagocytic syndromes. Hematology Am Soc Hematol Educ Program 2011;2011:178–183. DOI: 10.1182/asheducation-2011.1.178.
  6. Zoller EE, Lykens JE, Terrell CE, Aliberti J, Filipovich AH, Henson PM, et al. Hemophagocytosis causes a consumptive anemia of inflammation. J Exp Med 2011;208(6):1203–1214. DOI: 10.1084/jem.20102538.
  7. Goel S, Polski JM, Imran H. Sensitivity and specificity of bone marrow hemophagocytosis in hemophagocytic lymphohistiocytosis. Ann Clin Lab Sci 2012;42(1):21–25. PMID: 22371906.
  8. Li F, Yang Y, Jin F, Dehoedt C, Rao J, Zhou Y, et al. Clinical characteristics and prognostic factors of adult hemophagocytic syndrome patients: a retrospective study of increasing awareness of a disease from a single-center in China. Orphanet J Rare Dis 2015;10(1):20. DOI: 10.1186/s13023-015-0224-y.
  9. Bryceson YT, Pende D, Maul-Pavicic A, Gilmour KC, Ufheil H, Vraetz T, et al. A prospective evaluation of degranulation assays in the rapid diagnosis of familial hemophagocytic syndromes. Blood 2012;119(12):2754–2763. DOI: 10.1182/blood-2011-08-374199.
  10. Machowicz R, Janka G, Wiktor-Jedrzejczak W. Similar but not the same: differential diagnosis of HLH and sepsis. Crit Rev Oncol Hematol 2017;114:1–12. DOI: 10.1016/j.critrevonc.2017.03.023.
  11. Cascio A, Colomba C, Mililli D, Porto DL, Imburgia C, Iaria C. Tropical diseases in the ICU: please do not forget hemophagocytic lymphohistiocytosis. J Crit Care 2018;48:468–469. DOI: 10.1016/j.jcrc.2018.03.025.
  12. Gualdoni GA, Hofmann GA, Wohlfarth P, Winkler HM, Winkler S, Haslacher H, et al. Prevalence and outcome of secondary hemophagocytic lymphohistiocytosis among SIRS patients: results from a prospective cohort study. J Clin Med 2019;8(4):541. DOI: 10.3390/jcm8040541.
  13. Non LR, Patel R, Esmaeeli A, Despotovic V. Typhoid fever complicated by hemophagocytic lymphohistiocytosis and rhabdomyolysis. Am J Trop Med Hyg 2015;93(5):1068–1069. DOI: 10.4269/ajtmh.15-0385.
  14. La Rosée P, Horne A, Hines M, von Bahr Greenwood T, Machowicz R, Berliner N, et al. Recommendations for the management of hemophagocytic lymphohistiocytosis in adults. Blood 2019;133(23):2465–2477. DOI: 10.1182/blood.2018894618.
  15. Bergsten E, Horne A, Aricó M, Astigarraga I, Egeler RM, Filipovich AH, et al. Confirmed efficacy of etoposide and dexamethasone in HLH treatment: long-term results of the cooperative HLH-2004 study. Blood 2017;130(25):2728–2738. DOI: 10.1182/blood-2017-06-788349.
  16. Imashuku S, Kuriyama K, Teramura T, Ishii E, Kinugawa N, Kato M, et al. Requirement for etoposide in the treatment of Epstein-Barr virus–associated hemophagocytic lymphohistiocytosis. J Clin Oncol 2001;19(10):2665–2673. DOI: 10.1200/JCO.2001.19.10.2665.
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