Expression of Hemophagocytic Lymphohistiocytosis with Hepatic Dysfunction in a 50 days Old Infant

The Hemophagocytic Lymphohistiocytosis (HLH) is a rare disease with the pathologic hallmark of having aggressive proliferation of activated macrophages and histiocytes in the tissues and organs, which phagocyte other cells. The Incidence of HLH was estimated to be 1 case per 50000 – 300000. Case report: A 50 days infant was referred to Shahid Sadoughi Hospital with hepatic manifestations such as ascites, hepatosplenomegaly, markedly elevated liver function tests, herniated umbelical cord, abnormal coagulation profiles, and the bone marrow revealed HLH. Unfortunately the parents did not permit the appropriate treatment to be done and the infant died at age of 90 days old. since the HLH have nonspecific clinical signs, it should be considered as differential diagnosis of neonatal liver disease, especially when it is accompanied by cytopenias.