Background: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome characterized by pathologic macrophage activation. Clinical features include fever, cytopenia, hepatosplenomegaly, and coagulopathy. Liver dysfunction is often seen in HLH patients and occasionally leads to acute liver failure (ALF). There is limited data using liver transplantation (LT) to treat HLH associated ALF (HLH-ALF).
Methods: We present a pediatric HLH-ALF case treated with living donor liver transplantation from her mother and summarize the diagnosis and treatment.
Results: A 4-year-old girl was admitted to the hospital with intermittent fever for more than two months. Although the patient was diagnosed with Idiopathic thrombocytopenic purpura two years ago, work-up for infectious and autoimmune causes was negative. Differential diagnosis of lymphoma was considered but was eventually ruled out by lymph node biopsy. After antibiotic treatment, the patient's temperature dropped to normal, and was discharged. The patient had repeated illness outside the hospital and presented for recurrent high fever(40℃) with hepatosplenomegaly and superficial lymphadenopathy. The Symptomatic treatment was ineffective; the patient deteriorated rapidly with highly elevated transaminases and serum bilirubin, cytopenias, highly elevated ferritin and sCD25. Bone marrow biopsy revealed foamy macrophages engulfing mature and precursor erythrocytes, consistent with HLH. The patient's total bilirubin was continually increased and diagnosed secondary HLH-ALF, PELD scored 38. The patient was referred to our center for preparing liver transplantation. Considering the patient's critical condition, she received living donor liver transplantation from her mother (left lobe). The patient recovered well, the liver function improved and the jaundice gradually disappeared after liver transplantation.
Conclusion: The experience described here demonstrates the effect of LT in the treatment of HLH-ALF. Early investigation and treatment should be taken in HLH patients presenting with ALF. However, if liver dysfunction progresses rapidly, LT combined with posttransplant HLH-directed therapy offers the potential for improved survival.