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HLH Etoposide — SCE Medical Oncology MCQ

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HardOncological EmergenciesHLH EtoposideSCE Medical Oncology

A 62-year-old woman on pembrolizumab develops immune-related haemophagocytic lymphohistiocytosis (HLH). Despite high-dose steroids, her ferritin continues to rise (>25,000 µg/L), LDH is climbing, and she is pancytopenic. What additional therapy should be considered?

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Correct answer: B — Etoposide-based HLH protocol (as per HLH-2004) ± ruxolitinib — etoposide targets activated macrophages and is the standard escalation for steroid-refractory HLH; ruxolitinib (JAK1/2 inhibitor) dampens the cytokine storm

Steroid-refractory ICI-induced HLH/MAS is life-threatening (mortality >30%). Escalation: etoposide (targeting activated macrophages, per HLH-2004 protocol — typically 150 mg/m² weekly initially), ruxolitinib (JAK inhibitor dampening cytokine storm — emerging evidence from case series), IVIG and cyclosporin. ICU admission is usually required. Trigger identification (ICI discontinuation is mandatory) and haematology co-management are essential. The H-Score helps confirm HLH probability. Ferritin trajectory is the most useful monitoring biomarker.

Reference: ESMO 2024 irAE Management; HLH-2004 protocol; Sadaat et al Blood 2020