IMMUNOTHERAPY, cilt.17, sa.1, ss.11-17, 2025 (SCI-Expanded, Scopus)
ABSTRACT
Hemophagocytic Lymphohistiocytosis (HLH) is a severe and potentially life-threatening condition characterized
by an excessive and uncontrolled activation of the immune system. ICI-related hemophagocytic
lymphohistiocytosis (irHLH) is a rare immune-related adverse event with an incidence of 0.03% to 0.4%.
Although rare, it can be potentially lethal, with a high mortality rate of up to 50% in some cases. We
present a patient with recurrent glioblastoma who developed Hemophagocytic Lymphohistiocytosis s a
result of nivolumab treatment and was subsequently managed with cytokine-directed therapy (tocilizumab).
Early diagnosis and treatment of Hemophagocytic Lymphohistiocytosis (HLH) associated with
immune checkpoint inhibitors (ICIs) are indeed crucial due to the potentially life-threatening nature of
the condition.Cytokine-based treatments (such as anti-IL-6) may be appropriate for patients who do not
respond to high-dose steroids.
PLAIN LANGUAGE SUMMARY
This case report describes a 44-year-old man who experienced an immune-related adverse event,
specifically Hemophagocytic Lymphohistiocytosis (HLH), following immunotherapy for recurrent glioblastoma.
He initially presented with persistent fever and lethargy, and further testing confirmed the
immune checkpoint inhibitor-related event. High-dose dexamethasone and anti-IL-6 therapy (tocilizumab)
were administered, resulting in fever resolution and improvement in laboratory findings.
However, his clinical condition and disease progressively worsened. Unfortunately, he passed away
due to disease progression.