title: Hemophagocytic Lymphohistiocytosis, an Unclear Nosologic Entity: Case Report of an Adult Man with Rising of Amylase and Lipase and Spinal Cord Infiltration.
abstract: Here we present the case of a 57-years old patient affected by hemophagocytic lymphohistiocytosis (HLH), a rare disease characterized by an uncontrolled immune activation, resulting in clinical and biochemical manifestations of extreme inflammation. In a previous hospitalization, the patient showed fever, hepato-splenomegaly, pancytopenia, hyperferrtitinemia, lymphadenopathy and cholestasis. No diagnosis was done, however, he totally recovered after splenectomy. Eight months later, he relapsed, showing also hypofibrinogenemia, hypertriglyceridemia, hemophagocytic signs in bone marrow, cholestatic jaundice, high LDH and high PT-INR. Interestingly, he presented increased levels of amylase and lipase in absence of radiologic signs of pancreatitis. He was treated with Dexamethasone and Cyclosporine according to HLH-2004 guidelines. The clinical and biochemical manifestations disappeared in a few weeks, but he was newly hospitalized for lower limbs hypotonia caused by a hemophagocytic lesion of the cauda equina and lumbar cord. The death occurred in a few days, despite the immunosuppressive treatment.
authors.affiliation: Department of Interdisciplinary Medicine, University of Bari "Aldo Moro" , Bari, Italy.
authors.forename: Moris
authors.initials: M
authors.lastname: Sangineto
authors.affiliation: Department of Interdisciplinary Medicine, University of Bari "Aldo Moro" , Bari, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Perrone
authors.affiliation: Department of Interdisciplinary Medicine, University of Bari "Aldo Moro" , Bari, Italy.
authors.forename: Pasquale
authors.initials: P
authors.lastname: Agosti
authors.affiliation: Department of Interdisciplinary Medicine, University of Bari "Aldo Moro" , Bari, Italy.
authors.forename: Viera
authors.initials: V
authors.lastname: Boccuti
authors.affiliation: Department of Interdisciplinary Medicine, University of Bari "Aldo Moro" , Bari, Italy.
authors.forename: Anna
authors.initials: A
authors.lastname: Campobasso
authors.affiliation: Department of Interdisciplinary Medicine, University of Bari "Aldo Moro" , Bari, Italy.
authors.forename: Carlo
authors.initials: C
authors.lastname: Sabbà
keywords: Hemophagocytic Lymphohistiocytosis; amylase and lipase; spinal cord; splenectomy