Acute encephalopathy as a primary manifestation of haemophagocytic lymphohistiocytosis

摘要: Haemophagocytic lymphohistiocytosis (HLH) is characterized anatomically by an infiltration of multiple tissues with lymphocytes and haemophagocytic histiocytes. First symptoms are usually hepatosplenomegaly, pancytopenia, intractable fever. Up to 73% those HLH develop CNS involvement during the disease course. The peculiarity two patients presented here, a 20-month-old Italian female 4-year-old Moroccan female, that initial presenting neurological mimicked encephalitis, anticipating typical systemic 1 4 months. They developed progressive encephalopathy accompanied status epilepticus, one child secondary hydrocephalus. In both children it was not possible detect underlying infection or malignant there were no other cases in family suggested familial form HLH. Diagnosis initiation treatment delayed because encephalopathic clinical picture late onset features. As early diagnosis allows better therapeutical approaches, should be considered persistent findings encephalopathy, especially absence identification plausible pathogen.