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Hemophagocytic lymphohistiocytosis, an unclear nosologic entity: case report of an adult man with rising of amylase and lipase and spinal cord infiltration

Moris Sangineto, Antonio Perrone, Pasquale Agosti, Viera Boccuti, Anna Campobasso, Carlo Sabbà
  • Moris Sangineto
    Department of Interdisciplinary Medicine, University of Bari “Aldo Moro”, Italy | moris.sangineto@gmail.com
  • Antonio Perrone
    Department of Interdisciplinary Medicine, University of Bari “Aldo Moro”, Italy
  • Pasquale Agosti
    Department of Interdisciplinary Medicine, University of Bari “Aldo Moro”, Italy
  • Viera Boccuti
    Department of Interdisciplinary Medicine, University of Bari “Aldo Moro”, Italy
  • Anna Campobasso
    Department of Interdisciplinary Medicine, University of Bari “Aldo Moro”, Italy
  • Carlo Sabbà
    Department of Interdisciplinary Medicine, University of Bari “Aldo Moro”, Italy

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.

Keywords

Hemophagocytic Lymphohistiocytosis, spinal cord, amylase and lipase, splenectomy

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Submitted: 2016-09-02 10:24:06
Published: 2017-03-01 08:44:36
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Copyright (c) 2017 Moris Sangineto, Antonio Perrone, Pasquale Agosti, Viera Boccuti, Anna Campobasso, Carlo Sabbà

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