Fatal Hemophagocytic Syndrome : Three Autopsy Cases / 대한법의학회지

Hemophagocytic Syndrome (HS) is a systemic lymphohistiocytic proliferative disorder associated with infection or malignancies, particularly Epstein-Barr virus (EBV) HS is presented with high fever, skin rash, hepatosplenomegaly, and cytopenia. The characteristic pathologic finding is massive lymphohistiocytic infiltration with hemophagocytosis in various organs including bone marrow, liver, and spleen. Hyperproduction of cytokines by activated T lymphocytes has been presumed to account for the hemophagocytosis and clinical manifestations of HS. We report three childhood autopsy cases with HS which was confirmed by histopathologic examination. According to medical records, all cases had high fever with or without skin rash, cytopenia, AST/ALT elevation, and hyperfibrinogenemia, and showed multiple organ failure eventually. At autopsy, there were no specific gross findings except splenomegaly, but extensive lymphohistiocytic infiltration with hemophagocytosis was seen in various organs including lymph nodes, spleen, liver, and bone marrow. In one of three cases, EBV was identified with in situ hybridization method. As this disease has rapidly progressive clinical course with fatal outcome during childhood, so the possibility of HS should be considered in children presenting with high fever and hepatosplenomegaly.

A Case of Acute Adrenal Insufficiency with Bilateral Adrenal Hemorrhagic Infarction due to Ebstein-Barr Virus Infection in a Patient with Asymptomatic Chronic Adrenal Insufficiency / 대한내분비학회지

Acute adrenal insufficiency may result from adrenal crisis, hemorrhagic destruction, or the rapid withdrawal of steroids from patient with chronic steroid medication, congenital adrenal hyperplasia or those on other drugs. Acute hemorrhagic destruction of both adrenal glands can occur due to infection, trauma, anticoagulant therapy, antiphospholipid syndrome or a coagulation disorder. However, there have been no reports on acute hemorrhagic adrenal insufficiency due to the Ebstein-Barr virus (EBV). Herein, a case of acute adrenal insufficiency, with bilateral adrenal hemorrhagic infarction, is reported in a patient with asymptomatic chronic adrenal insufficiency. A 42-year-old man presented with general weakness, weight loss and hyperpigmentation of several months duration. He suffered from a sore throat, general myalgia and a headache on admission. The laboratory findings were lymphocytosis, positive EBV IgM antibody, low cortisol level and a high level of adrenocorticotropic hormone (ACTH). Adrenocortical autoantibody and PCR for Mycobacterium tuberculosis showed negative findings. The serologic findings for CMV and HIV were negative. Fine needle aspiration of the adrenal gland revealed a hemorrhagic infarction and positive staining for the anti-EBV antibody. Acute adrenal insufficiency was then diagnosed with a bilateral adrenal hemorrhagic infarction due to the EBV infection in the patient, also with asymptomatic chronic adrenal insufficiency. This is the first case of acute adrenal insufficiency with bilateral hemorrhagic infarction, due to an EBV infection