Successful Management of Suspected Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis in BRAF-Mutant Cholangiocarcinoma Following Treatment With Immune Checkpoint Inhibitors and BRAF / MEK Inhibitors: A Case Report
Hemophagocytic lymphohistiocytosis (HLH) is a frequent complication only 3 months after the initiation of BRAF/MEK therapy in BRAF-mutated cholangiocarcinoma with a history of ICI therapy.
A 49‑year‑old man with BRAF V600E‑mutant intrahepatic cholangiocarcinoma developed Epstein‑Barr virus (EBV)‑associated hemophagocytic lymphohistiocytosis (HLH) after sequential therapy with a durvalumab (an immune checkpoint inhibitor, ICI) and dabrafenib plus trametinib (BRAF/MEK inhibitors). HLH manifested as fever, thrombocytopenia, hyper‑ferritinemia, and high soluble interleukin‑2 receptor levels. Prompt diagnosis and high‑dose methylprednisolone pulse therapy followed by oral prednisolone led to rapid clinical improvement and survival. The case illustrates that HLH can be a fatal adverse event after ICI and BRAF/MEK inhibitor therapy, especially in the setting of EBV reactivation.
HLH can present as severe sepsis and may be under‑diagnosed after ICI or BRAF/MEK therapy. Early recognition and steroid therapy proved lifesaving in this patient.
Evidence level: Klinikai vizsgálat. Formális klinikai vizsgálati eredmény.
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