Successful management of adult hemophagocytic lymphohistiocytosis with an etoposide-containing regimen: Case report and literature review

Objective: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder characterized by a hyperimmune response. Although HLH is well described in the pediatric population, less is known about the appropriate management in adults. Despite the inclusion of etoposide in HLH society protocols, some clinicians refrain from using it due to its cytotoxicity and potential adverse effects. Case: We report here the case of a 43-year-old male presenting with fever and pancytopenia, who was diagnosed with HLH following further evaluation. The patient immediately started the HLH-2004 initial protocol, which consisted of dexamethasone, etoposide, cyclosporine, and IVIG. Symptoms improved after 2 weeks of treatment. Subsequent bone marrow biopsy showed normal results, and the patient achieved complete remission, leading to the termination of treatment in the 12th week of the protocol. At 8 months post-treatment, the patient remained recurrence-free with normal hematological and biochemical test results. Conclusion: This case underscores the importance of considering etoposide-containing regimens, particularly for medically fit patients.