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.

Acute necrotizing encephalopathy associated with lymphoma-associated hemophagocytic lymphohistiocytosis: A case report and literature review

“…HLH is associated with a series of pathological changes due to the overactivation of the body’s macrophages, lymphocytes, and other immune cells. The common causes of HLH include genetic susceptibility, viral infections, autoimmune diseases, and malignancies, including lymphomas ( 2 ). The clinical manifestations, laboratory tests, and imaging examinations of this child meet the diagnostic criteria of HLH ( 8 ).…”

“…Moreover, there is a massive release of inflammatory factors accompanying hemophagocytosis of tissues and organs, including the primary and secondary categories ( 1 ). Lymphoma-associated hemophagocytic lymphohistiocytosis (LA-HLH) is a common cause of secondary HLH ( 2 ). Acute necrotizing encephalopathy (ANE) is considered one of the most critical subtypes of acute encephalopathy ( 3 ).…”