Introduction

Macrophage activation syndrome (MAS), also known as secondary hemophagocytic lymfohistiocytosis (HLH) is poorly described life-threatening condition. It is caused by excessive activation of immune system with subsequent proinflammatory state resulting in multiorgan involvement. Clinically, MAS is characterized by fever, cytopenia, hepatosplenomegaly and elevated circulating ferritin. MAS can develop into acute liver failure (ALF) with poor prognosis.

Aims & Methods

We retrospectively analysed patients referred to our transplantation centre for ALF in whom the diagnosis of MAS was confirmed by histology. We focused on diagnosis leading to MAS, survival, transplantation outcome and clinical, laboratory and histological findings.

Results

From January 2005 to November 2022, we found eight patients with MAS referred to our centre for ALF. The underlying condition was EBV infection in five cases and adult Still disease in three cases. All eight patients had strongly elevated circulating ferritin (in average 19321 μg/l), leukocytosis and thrombocytopenia. All patients except one had at least one negative bone marrow or lymph node examination prior to definitive diagnosis of MAS. Seven patients were treated with high-dose corticosteroids. Four patients met King's College criteria for urgent liver transplantation. Two of them were transplanted. Despite intensive therapy based on mechanical ventilation, continuous renal replacement therapy and circulation support, their clinical condition further deteriorated and all four patients died on average 34 days after symptom onset of multiorgan failure. At the autopsy, hemophagocytic macrophages have been described in multiple organs. Four patients who responded to corticosteroid therapy are in long-term remission after specialized haematological treatment with etoposide.

Conclusion

Macrophage activation syndrome represents rare cause of acute liver failure with high mortality, it is difficult to diagnose and even harder to treat. Our case series suggests that when the patients meet King's College criteria, they die despite the successful liver transplant. Therefore, it is important to carefully consider liver transplantation in these patients.