Exploring the Complexities of Liver Transplantation for Secondary Hemophagocytic Lymphohistiocytosis-Associated Acute Liver Failure.

Background: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome characterized by the aberrant activation of macrophages. Liver dysfunction is often observed in patients with HLH and has the potential to advance to acute liver failure (ALF). However, limited data exist regarding the application of liver transplantation (LTx) for the management of HLH-associated acute liver failure (HLH-ALF).

Methods: A retrospective analysis was conducted on a pediatric case of LTx for HLH-ALF at our center, and previously documented cases of liver transplantation for this condition were summarized.

Results: A 4-year-old girl was admitted with intermittent fever that had persisted for more than 2 months. She exhibited recurrent high fever (40°C), accompanied by hepatosplenomegaly and superficial lymphadenopathy. The patient's condition rapidly progressed and fulfilled the HLH-2004 diagnostic criteria. The liver function progressively deteriorated. The patient was diagnosed with secondary HLH-ALF. Given the patient's critical condition, she underwent a living donor liver transplantation from her mother (left lobe). Despite experiencing rejection and human herpes virus 6 (HHV 6) infection, the patient recovered well and achieved stable disease.

Conclusion: This experience underscores the potential effectiveness of LTx in the management of HLH-ALF. Prompt investigation and intervention are crucial for patients with HLH exhibiting ALF. Although complex post-transplantation complications may arise, LTx remains a viable treatment option for HLH-ALF.