J Rheum Dis.  2024 Apr;31(2):125-129. 10.4078/jrd.2023.0027.

Successful treatment of hemophagocytic lymphohistiocytosis in a patient with systemic lupus erythematosus with ruxolitinib: a case report

Affiliations
  • 1Division of Rheumatology, Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea
  • 2Division of Rheumatology, Department of Internal Medicine, Hallym University Dongtan Sacred Heart Hospital, Hwaseong, Korea

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hematological disorder characterized by uncontrolled activation of CD8+ T and natural killer cells, leading to a cytokine storm and severe organ dysfunction. Although secondary HLH related to autoimmune diseases usually demonstrates a good treatment response to immunosuppressive therapy for underlying conditions, there is no consensus regarding the treatment in case of unresponsiveness to the treatment. Herein, we present a case of HLH that was unresponsive to high-dose glucocorticoid and cyclosporine treatment in a patient with newly diagnosed systemic lupus erythematosus. The patient’s clinical features and laboratory abnormalities rapidly improved with ruxolitinib, an oral Janus kinase 1 and 2 (JAK1/2) inhibitor. This result suggests that blocking JAK-STAT pathway may be a potential treatment option in patients with refractory HLH secondary to autoimmune diseases.

Keyword

Hemophagocytic lymphohistiocytosis; Janus kinase inhibitors; Ruxolitinib

Figure

  • Figure 1 Summary of immunosuppressive treatment. HLH: hemophagocytic lymphohistiocytosis, CMV: cytomegalovirus, mPD: methylprednisolone, PD: prednisolone.

  • Figure 2 Change in laboratory findings before and after the ruxolitinib treatment. Dashed lines in the graphs indicate the reference range of each laboratory value. HLH: hemophagocytic lymphohistiocytosis, WBC: white blood cell, GFR: glomerular filtration rate, ALT: alanine transferase.


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