A case of eculizumab-induced hemophagocytic lymphohistiocytosis (HLH) in a myasthenia gravis patient

Authors

  • Salma S Elkolaly University of Missouri
  • Dinanath P Attele University of Utah
  • Erik Ensrud University of Missouri

DOI:

https://doi.org/10.17161/rrnmf.v6i2_2025.23412

Keywords:

hemophagocytic lymphohistiocytosis, myasthenia gravis, eculizumab, antibodies, monoclonal, humanized

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is an immune hyperactivation state that can occur in immunosuppressed patients and is associated with high mortality and worse prognosis. We present a case of 78-year-old patient on multiple immune suppressing medications, including eculizumab and azathioprine for myasthenia gravis, who presented to our hospital for evaluation of hyperbilirubinemia. She had extensive laboratory workup that was significant for anemia, thrombocytopenia, increased ferritin level, and hyponatremia. Additionally, she had increased CD25 and CXCL9 leading to the diagnosis of HLH. Investigations for triggering factors identified eculizumab after excluding multiple infectious and rheumatologic conditions. Unfortunately, the patient did not survive. We recommend evaluating for high ferritin as a reliable predictor for HLH for patients on myasthenia gravis on eculizumab.

Downloads

Download data is not yet available.

References

1. Filipovich A, McClain K, Grom A. Histiocytic Disorders: Recent Insights into Pathophysiology and Practical Guidelines. Biol Blood Marrow Transplant [Internet]. 2010 Jan 1;16(1):S82–9. Available from: https://doi.org/10.1016/j.bbmt.2009.11.014

2. Tabaja H, Kanj A, El Zein S, Comba IY, Chehab O, Mahmood M. A Review of Hemophagocytic Lymphohistiocytosis in Patients With HIV. Open forum Infect Dis. 2022 Apr;9(4):ofac071.

3. Bode SF, Ammann S, Al-Herz W, Bataneant M, Dvorak CC, Gehring S, et al. The syndrome of hemophagocytic lymphohistiocytosis in primary immunodeficiencies: implications for differential diagnosis and pathogenesis. Haematologica. 2015 Jul;100(7):978–88.

4. Kennedy LB, Salama AKS. A review of cancer immunotherapy toxicity. CA Cancer J Clin [Internet]. 2020 Mar 1;70(2):86–104. Available from: https://doi.org/10.3322/caac.21596

5. Howard Jr JF, Utsugisawa K, Benatar M, Murai H, Barohn RJ, Illa I, et al. Safety and efficacy of eculizumab in anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis (REGAIN): a phase 3, randomised, double-blind, placebo-controlled, multicentre study. Lancet Neurol [Internet]. 2017 Dec 1;16(12):976–86. Available from: https://doi.org/10.1016/S1474-4422(17)30369-1

6. Rother RP, Rollins SA, Mojcik CF, Brodsky RA, Bell L. Discovery and development of the complement inhibitor eculizumab for the treatment of paroxysmal nocturnal hemoglobinuria. Nat Biotechnol. 2007 Nov;25(11):1256–64.

7. Dhillon S. Eculizumab: A Review in Generalized Myasthenia Gravis. Drugs. 2018 Mar;78(3):367–76.

8. Gloude NJ, Dandoy CE, Davies SM, Myers KC, Jordan MB, Marsh RA, et al. Thinking Beyond HLH: Clinical Features of Patients with Concurrent Presentation of Hemophagocytic Lymphohistiocytosis and Thrombotic Microangiopathy. J Clin Immunol. 2020 Jul;40(5):699–707.

9. Gupta S, Weitzman S. Primary and secondary hemophagocytic lymphohistiocytosis: clinical features, pathogenesis and therapy. Expert Rev Clin Immunol. 2010 Jan;6(1):137–54.

10. Allen CE, Yu X, Kozinetz CA, McClain KL. Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2008 Jun;50(6):1227–35.

11. Basu S, Maji B, Barman S, Ghosh A. Hyperferritinemia in Hemophagocytic Lymphohistiocytosis: A Single Institution Experience in Pediatric Patients. Indian J Clin Biochem. 2018 Jan;33(1):108–12.

12. Grangé S, Buchonnet G, Besnier E, Artaud-Macari E, Beduneau G, Carpentier D, et al. The Use of Ferritin to Identify Critically Ill Patients With Secondary Hemophagocytic Lymphohistiocytosis. Crit Care Med. 2016 Nov;44(11):e1045–53.

Downloads

Published

2025-08-04

Issue

Section

Clinic and Case Reports

How to Cite

Elkolaly, S., Attele, D., & Ensrud, E. (2025). A case of eculizumab-induced hemophagocytic lymphohistiocytosis (HLH) in a myasthenia gravis patient. RRNMF Neuromuscular Journal, 6(2). https://doi.org/10.17161/rrnmf.v6i2_2025.23412