Haemophagocytic lymphohistiocytosis (HLH) is a rare disease with a sepsis-like progression that leads to multiple organ dysfunction syndrome, especially in preterm infants. We present herein a case of HLH in a premature infant presenting with disseminated intravascular coagulopathy (DIC) and liver failure. A male infant, with weight 810g and delivered at the gestational age of 25 weeks and 2 days, was misdiagnosed with tyrosinaemia for several weeks. He presented with anaemia, thrombocytopaenia, persistent DIC, and elevated liver enzymes despite continuous transfusion and broad-spectrum antibiotics. A bone marrow puncture biopsy revealed haemophagocytosis, leading to HLH diagnosis. It is important for paediatricians to consider the possibility of HLH when liver function test results are abnormal in such patients.
Keywords: Hemophagocytic lymphohistiocytosis, Liver failure, Premature infant..