Case Report: Hemophagocytic lymphohistiocytosis masquerading as drug-induced liver injury: successful treatment with low-dose ruxolitinib and glucocorticoids - Summary - MDSpire

Case Report: Hemophagocytic lymphohistiocytosis masquerading as drug-induced liver injury: successful treatment with low-dose ruxolitinib and glucocorticoids

  • By

  • Yang Dai

  • Luocheng Zhang

  • Xushu Zhong

  • Ailin Zhao

  • Ting Niu

  • May 28, 2026

  • 0 min

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Objective:

To describe the clinical features, treatment, and outcomes of three adult patients presenting with DILI-mimicking HLH and evaluate the effectiveness of low-dose ruxolitinib combined with corticosteroids.

Key Findings:
  • All patients responded rapidly to the combination therapy, with progressive normalization of bilirubin and inflammatory markers.
  • Transient declines in blood counts occurred but resolved without intervention.
  • Genetic testing revealed heterozygous variants in immune-regulatory genes classified as variants of uncertain significance.
Interpretation:

HLH can initially present as severe DILI, posing diagnostic challenges. Short-course, low-dose ruxolitinib combined with corticosteroids is a safe and effective treatment in patients with hepatic-predominant HLH.

Limitations:
  • The study is based on a small sample size of three patients.
  • Genetic variants identified were classified as variants of uncertain significance, limiting definitive conclusions about their role.
Conclusion:

Early recognition and timely immunomodulatory therapy may improve outcomes in this life-threatening but treatable condition.

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