Skip to main content

Hemophagocytic Lymphohistiocytosis

  • Chapter
  • First Online:
Evidence-Based Critical Care

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder of pathologic immune system activation. Patients with HLH, however, often present critically ill, and HLH should be considered in cases of sepsis-like illness which recur or do not respond to standard treatment. While treatment of HLH with chemotherapy and immunosuppression should be guided by an expert hematologist, the diagnosis is often made by the critical care medicine practitioner. We present a case illustrating the challenges of defining this syndrome in a patient with recurrent critical illness, and review the evidence underlying diagnostic and prognostic criteria for this protean syndrome.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 149.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 199.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Similar content being viewed by others

References

  1. Janka GE, Lehmberg K. Hemophagocytic syndromes–an update. Blood Rev. [Internet]. 2014 [Cited 27 May 2014 ]; Available from: http://www.sciencedirect.com/science/article/pii/S0268960X14000289.

  2. Jordan MB, Allen CE, Weitzman S, Filipovich AH, McClain KL. How I treat hemophagocytic lymphohistiocytosis. Blood [Internet]. 2011 [Cited 27 May 2014];118:4041–52. Available from: http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=3204727&tool=pmcentrez&rendertype=abstract.

  3. Wang Y, Wang Z, Zhang J, Wei Q, Tang R, Qi J, et al. Genetic features of late onset primary hemophagocytic lymphohistiocytosis in adolescence or adulthood. PLoS One [Internet]. 2014 [Cited 16 Jan 2015];9:e107386. Available from: http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=4169386&tool=pmcentrez&rendertype=abstract.

  4. Li J, Wang Q, Zheng W, Ma J, Zhang W, Wang W, et al. Hemophagocytic lymphohistiocytosis: clinical analysis of 103 adult patients. Medicine (Baltimore). [Internet]. 2014 [Cited 13 Jun 2014];93:100–5. Available from: http://www.ncbi.nlm.nih.gov/pubmed/24646466.

  5. Ramos-Casals M, Brito-Zerón P, López-Guillermo A, Khamashta MA, Bosch X. Adult haemophagocytic syndrome. Lancet [Internet]. 2014 [Cited 28 May 2014];383:1503–16. Available from: http://www.sciencedirect.com/science/article/pii/S014067361361048X.

  6. 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 [Internet]. 2007 [Cited 31 May 2014];48:124–31. Available from: http://www.ncbi.nlm.nih.gov/pubmed/16937360.

  7. Goel S, Polski JM, Imran H. Sensitivity and specificity of bone marrow hemophagocytosis in hemophagocytic lymphohistiocytosis. Ann Clin Lab Sci. [Internet]. 2012 [Cited 13 Jun 2014];42:21–5. Available from: http://www.ncbi.nlm.nih.gov/pubmed/22371906.

  8. Henter JI, Samuelsson-Horne A, Aricò M, Egeler RM, Elinder G, Filipovich AH, et al. Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation. Blood [Internet]. 2002 [Cited 16 Jun 2014];100:2367–73. Available from: http://www.ncbi.nlm.nih.gov/pubmed/12239144.

  9. Trottestam H, Horne A, Aricò M, Egeler RM, Filipovich AH, Gadner H, et al. Chemoimmunotherapy for hemophagocytic lymphohistiocytosis: long-term results of the HLH-94 treatment protocol. Blood [Internet]. 2011 [Cited 27 May 2014];118:4577–84. Available from: http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=3208276&tool=pmcentrez&rendertype=abstract.

  10. Han A-R, Lee HR, Park BB, Hwang IG, Park S, Lee SC, et al. Lymphoma-associated hemophagocytic syndrome: clinical features and treatment outcome. Ann Hematol. [Internet]. 2007 [Cited 16 Jun 2014];86:493–8. Available from: http://www.ncbi.nlm.nih.gov/pubmed/17347847.

  11. Yu JT, Wang CY, Yang Y, Wang RC, Chang KH, Hwang WL, et al. Lymphoma-associated hemophagocytic lymphohistiocytosis: experience in adults from a single institution. Ann Hematol. [Internet]. 2013 [Cited 13 Jun 2014];92:1529–36. Available from: http://www.ncbi.nlm.nih.gov/pubmed/23700280.

  12. Rivière S, Galicier L, Coppo P, Marzac C, Aumont C, Lambotte O, et al. Reactive hemophagocytic syndrome in adults: a multicenter retrospective analysis of 162 patients. Am J Med. [Internet]. 2014 [Cited 23 May 2014]; Available from: http://www.ncbi.nlm.nih.gov/pubmed/24835040.

  13. Otrock ZK, Eby CS. Clinical characteristics, prognostic factors and outcomes of adult patients with hemophagocytic lymphohistiocytosis. Am J Hematol. [Internet]. 2014 [Cited 16 Jan 2015];00:1–5. Available from: http://www.ncbi.nlm.nih.gov/pubmed/25469675.

  14. Buyse S, Teixeira L, Galicier L, Mariotte E, Lemiale V, Seguin A, et al. Critical care management of patients with hemophagocytic lymphohistiocytosis. Intensive Care Med. [Internet]. 2010 [Cited 7 Jun 2014];36:1695–702. Available from: http://www.ncbi.nlm.nih.gov/pubmed/20532477.

  15. Allen CE, Yu X, Kozinetz CA, McClain KL. Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer [Internet]. 2008 [Cited 16 Jun 2014];50:1227–35. Available from: http://www.ncbi.nlm.nih.gov/pubmed/18085676.

  16. Lin TF, Ferlic-Stark LL, Allen CE, Kozinetz CA, McClain KL. Rate of decline of ferritin in patients with hemophagocytic lymphohistiocytosis as a prognostic variable for mortality. Pediatr Blood Cancer [Internet]. 2011 [Cited 14 Jun 2014];56:154–5. Available from: http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=3444147&tool=pmcentrez&rendertype=abstract.

  17. Tothova Z, Berliner N. Hemophagocytic syndrome and critical illness: new insights into diagnosis and management. J Intensive Care Med. [Internet]. 2014 [Cited 13 Jun 2014]; Available from: http://www.ncbi.nlm.nih.gov/pubmed/24407034.

  18. Schram AM, Campigotto F, Mullally A, Fogerty A, Massarotti E, Neuberg D, et al. Marked hyperferritinemia does not predict for HLH in the adult population. Blood [Internet]. 2015 [Cited 16 Jan 2015];1–19. Available from: http://www.ncbi.nlm.nih.gov/pubmed/25573993.

  19. Tsuji T, Hirano T, Yamasaki H, Tsuji M, Tsuda H. A high sIL-2R/ferritin ratio is a useful marker for the diagnosis of lymphoma-associated hemophagocytic syndrome. Ann Hematol. [Internet]. 2014 [Cited 13 Jun 2014];93:821–6. Available from: http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=3976506&tool=pmcentrez&rendertype=abstract.

  20. Xu XJ, Tang YM, Song H, Yang SL, Xu WQ, Zhao N, et al. Diagnostic accuracy of a specific cytokine pattern in hemophagocytic lymphohistiocytosis in children. J. Pediatr. [Internet]. 2012 [Cited 27 May 2014];160:984–90.e1. Available from: http://www.ncbi.nlm.nih.gov/pubmed/22226576.

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Benjamin H. Singer .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2017 Springer International Publishing Switzerland

About this chapter

Cite this chapter

Singer, B.H., Loomis-King, H.A. (2017). Hemophagocytic Lymphohistiocytosis. In: Hyzy, R. (eds) Evidence-Based Critical Care. Springer, Cham. https://doi.org/10.1007/978-3-319-43341-7_73

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-43341-7_73

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-43339-4

  • Online ISBN: 978-3-319-43341-7

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics