Author/Authors :
Gülen, H Celal Bayar Üniversitesi - Tıp Fakültesi - Pediatrik Hematoloji Bilim Dalı, Turkey , Aslan, S Celal Bayar Üniversitesi - Tıp Fakültesi - Çocuk Saglıgı ve Hastalıkları Anabilim Dalı, Turkey , Simsek, A Celal Bayar Üniversitesi - Tıp Fakültesi - Çocuk Saglıgı ve Hastalıkları Anabilim Dalı, Turkey , Ayhan, S Celal Bayar Üniversitesi - Tıp Fakültesi - Patoloji Anabilim Dalı, Turkey , Moumin, N Celal Bayar Üniversitesi - Tıp Fakültesi - Çocuk Saglıgı ve Hastalıkları Anabilim Dalı, Turkey , Kasirga, E Celal Bayar Üniversitesi - Tıp Fakültesi - Pediatrik Gastroenteroloji Bilim Dalı, Turkey
Abstract :
Hemophagocytic lymphohistiocytosis (HLH) is an uncommon clinical entity characterized by fever, pancytopenia, hepatosplenomegaly and hemophagocytosis in bone marrow, liver or lymph nodes. Fulminant hepatic failure (FHF) is a clinical syndrome resulting from massive necrosis of hepatocytes or severe functional impairment of hepatocytes in a patient who does not have a pre-existing liver disease. Hepatic manifestations are common in hemophagocytic lymphohistiocytosis and overt hepatic failure may occur, but initial presentation as fulminant hepatic failure is not well recognized. We report the case of a 9 year old girl presented with fulminant hepatic failure and “Hemophagocytic lymphohistiocytosis” should be considered in the differential diagnosis of fulminant hepatic failure in children.
