Journal of Trauma and Injury

The Korean Society of Traumatology (대한외상학회)
권호 표지
DOI QR코드

DOI QR Code

Successful treatment of hemophagocytic lymphohistiocytosis in a trauma patient: a case report

  • Young Soo Chung (Department of Surgery, Dong-A University Hospital, Dong-A University College of Medicine) ;
  • Jihoon Kim (Department of Trauma Surgery, Ulsan University Hospital, University of Ulsan College of Medicine)
  • Received : 2024.12.10
  • Accepted : 2025.01.02
  • Published : 2025.03.31
Download PDF
⟨ Previous Next ⟩

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory syndrome characterized by excessive activation of the immune system. This case report describes an unusual presentation of HLH triggered by severe trauma from a motorcycle accident, which is a departure from traditional associations with infections, malignancies, or autoimmune conditions. A 40-year-old man with multiple traumatic injuries developed persistent fever, pancytopenia, and elevated inflammatory markers following orthopedic surgery. Despite empiric antibiotic therapy, his condition deteriorated, exhibiting high fever, skin rash, hepatic dysfunction, and marked elevation of ferritin levels (32,901 ng/mL). Bone marrow biopsy confirmed the diagnosis of HLH, and treatment was initiated according to the HLH-2004 protocol, which included methylprednisolone, intravenous immunoglobulin, and immunosuppressive therapy. The patient demonstrated significant clinical improvement and was discharged after 37 days, with no recurrence observed during the follow-up period. This case underscores the need to consider HLH in trauma patients presenting with unexplained inflammatory responses and illustrates that prompt diagnosis and aggressive treatment can lead to successful outcomes.

Keywords

References

  1. Janka G. Hemophagocytic lymphohistiocytosis: when the immune system runs amok. Klin Padiatr 2009;221:278–85. https://doi.org/10.1055/s-0029-1237386
  2. La Rosee P, Horne A, Hines M, et al. Recommendations for the management of hemophagocytic lymphohistiocytosis in adults. Blood 2019;133:2465–77. https://doi.org/10.1182/blood.2018894618
  3. Schram AM, Comstock P, Campo M, et al. Haemophagocytic lymphohistiocytosis in adults: a multicentre case series over 7 years. Br J Haematol 2016;172:412–9. https://doi.org/10.1111/bjh.13837
  4. George MR. Hemophagocytic lymphohistiocytosis: review of etiologies and management. J Blood Med 2014;5:69–86. https://doi.org/10.2147/JBM.S46255
  5. Knaak C, Nyvlt P, Schuster FS, et al. Hemophagocytic lymphohistiocytosis in critically ill patients: diagnostic reliability of HLH-2004 criteria and HScore. Crit Care 2020;24:244. https://doi.org/10.1186/s13054-020-02941-3
  6. Henter JI, Horne A, Arico M, et al. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007;48:124–31. https://doi.org/10.1002/pbc.21039
  7. Ramos-Casals M, Brito-Zeron P, Lopez-Guillermo A, Khamashta MA, Bosch X. Adult haemophagocytic syndrome. Lancet 2014;383:1503–16. https://doi.org/10.1016/S0140-6736(13)61048-X
  8. Ishii E, Ohga S, Imashuku S, et al. Nationwide survey of hemophagocytic lymphohistiocytosis in Japan. Int J Hematol 2007;86:58–65. https://doi.org/10.1532/IJH97.07012
  9. Parikh SA, Kapoor P, Letendre L, Kumar S, Wolanskyj AP. Prognostic factors and outcomes of adults with hemophagocytic lymphohistiocytosis. Mayo Clin Proc 2014;89:484–92. https://doi.org/10.1016/j.mayocp.2013.12.012
  10. Yildiz H, Van Den Neste E, Defour JP, Danse E, Yombi JC. Adult haemophagocytic lymphohistiocytosis: a review. QJM 2022;115:205–13. https://doi.org/10.1093/qjmed/hcaa011
  11. Rosario C, Zandman-Goddard G, Meyron-Holtz EG, D'Cruz DP, Shoenfeld Y. The hyperferritinemic syndrome: macrophage activation syndrome, Still's disease, septic shock and catastrophic antiphospholipid syndrome. BMC Med 2013;11:185. https://doi.org/10.1186/1741-7015-11-185
  12. Allen CE, Yu X, Kozinetz CA, McClain KL. Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2008;50:1227–35. https://doi.org/10.1002/pbc.21423
  13. Fisman DN. Hemophagocytic syndromes and infection. Emerg Infect Dis 2000;6:601–8. https://doi.org/10.3201/eid0606.000608
  14. Filipovich AH, Chandrakasan S. Pathogenesis of hemophagocytic lymphohistiocytosis. Hematol Oncol Clin North Am 2015;29:895–902. https://doi.org/10.1016/j.hoc.2015.06.007
  15. Henderson LA, Canna SW, Schulert GS, et al. On the alert for cytokine storm: immunopathology in COVID-19. Arthritis Rheumatol 2020;72:1059–63. https://doi.org/10.1002/art.41285