Familial haemophagocytic lymphohistiocytosis: rare cause of acute liver failure in a neonate--a case report

Abstract

Familial haemophagocytic lymphohistiocytosis (FHLH) is a rare lifethreatening disorder of the immune system characterized by proliferation and infiltration of hyperactivated macrophages and T-lymphocytes and manifesting as acute illness with prolonged fever, cytopenias, and hepatosplenomegaly. We describe a one-month-old female with FHLH and rapidly progressing liver failure. To conclude, FHLH should be included in the differential diagnosis of acute liver failure in neonates and young infants.

Keywords: Familial haemophagocytic lymphohistiocytosis; Acute liver failure; Neonate; Case report