Correct!
5. All of the above

In many cases of HLH, an underlying infection or malignancy requires specific therapy. Non-specific immunotherapy of HLH typically involves the use of corticosteroids, intravenous immunoglobulin G (IVIg) and etoposide (7). However, etoposide, a myelosuppressive chemotherapy agent, often causes severe pancytopenia in critically ill patients with hepatic and/or renal dysfunction typical of sepsis (7).

Anakinra (Kineret®) is a recombinant modified human interleukin 1 receptor antagonist used to treat rheumatoid arthritis. Increasing evidence suggests efficacy and safety of anakinra in both children and adults with HLH (8). In this issue of SWJPCC, Henry and Raschke (9) add to the mounting observational evidence of the efficacy of anakinra in adults reporting a case series of 7 adult patients with 71% survival.

The patient described above was treated with corticosteroids, IVIg and anakinra and gradually recovered from her illness over a period of months.

References

  1. Henter J, Horne A, Aricó M, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007;48:124-31. [CrossRef] [PubMed]
  2. Hayden A, Lin M, Park S, Pudek M, Schneider M, Jordan MB, Mattman A, Chen LYC. Soluble interleukin-2 receptor is a sensitive diagnostic test in adult HLH. Blood Adv. 2017 Dec 6;1(26):2529-34. [CrossRef] [PubMed]
  3. Stéphan F, Thiolière B, Verdy E, Tulliez M. Role of hemophagocytic histiocytosis in the etiology of thrombocytopenia in patients with sepsis syndrome or septic shock. Clin Infect Dis. 1997 Nov;25(5):1159-64. [CrossRef] [PubMed]
  4. Raschke RA, Garcia-Orr R. Hemophagocytic lymphohistiocytosis: A potentially underrecognized association with systemic inflammatory response syndrome, severe sepsis, and septic shock in adults. Chest 2011;140:933-8. [CrossRef] [PubMed]
  5. Strauss R, Neureiter D, Westenburger B, Wehler M, Kirchner T, Hahn EG. Multifactorial risk analysis of bone marrow histiocytic hyperplasia with hemophagocytosis in critically ill medical patients--a postmortem clinicopathologic analysis. Crit Care Med. 2004 Jun;32(6):1316-21. [CrossRef] [PubMed]
  6. Beutel G, Wiesner O, Eder M, et al. Virus-associated hemophagocytic syndrome as a major contributor to death in patients with 2009 influenza A (H1N1) infection. Crit Care. 2011;15(2):R80. [CrossRef] [PubMed]
  7. Wohlfarth P, Agis H, Gualdoni GA, Weber J, Staudinger T, Schellongowski P, Robak O. Interleukin 1 receptor antagonist anakinra, intravenous immunoglobulin, and corticosteroids in the management of critically ill adult patients with hemophagocytic lymphohistiocytosis. J Intensive Care Med. 2017 Jan 1:885066617711386. [CrossRef] [PubMed]
  8. Rajesekaran S, Kruse K, Kovey K, et al. Therapeutic role of anakinra, an interleukin-1 receptor antagonist, in the management of secondary hemophagocytic lymphohistiocytosis/sepsis/multiple organ dysfunction/macrophage activating syndrome in critically ill children. Pediatr Crit Care Med. 2014;15:401-8. [CrossRef] [PubMed]
  9. Henry K, Raschke RA. An observational study demonstrating possible efficacy of interleukin-1 antagonist (anakinra) in critically-ill patients with hemophagocytic lymphohistiocytosis. Southwest J Pulm Crit Care. 2019;18(6):177-86. [CrossRef]  

Home/Critical Care