title: A case of hemophagocytic lymphohistiocytosis after the primary Epstein-Barr virus infection.
abstract: The Epstein-Barr virus (EBV) infection is known to result in infectious mononucleosis, hemophagocytic syndrome, chronic active EBV infection, and lymphoma. Among them, hemophagocytic syndrome sometimes causes thrombocytopenia, which is often life threatening because of its hemorrhagic complications such as gastrointestinal bleeding and pulmonary alveolar hemorrhage. A young adult case of critical hemophagocytic syndrome after primary EBV infection is presented. Chemotherapy was performed using methyl prednisolone succinate, prednisolone, cyclosporin A, and 20 mg/kg of cyclophosphamide. The patient received intensive care, including plasma exchange for hepatic failure, extracorporeal membrane oxygenation for acute respiratory failure, and splenectomy for hemophagocytosis; however, the patient died of multiple organ failure, including fulminant hepatic failure. The pathologic examination of the resected specimen demonstrated infiltrated macrophages containing many phagocytosed erythrocytes. Further immunopathologic examination of these cells showed that the histiocyte markers were positive, whereas the T-cell marker was negative. In view of these findings, definite diagnosis of EBV-related hemophagocytic lymphohistiocytosis could not be made at that time. The immunohistologic examinations on the liver necropsy specimen provided the evidences suggesting the morbid activation of the hepatic stellate macrophage by EBV-infected T/NK cells and subsequent apoptosis induction of the liver cells through the Fas ligand pathway.
authors.affiliation: Department of Emergency and Critical Care Medicine, Kansai Medical University Hirakata Hospital, Hirakata-city, Osaka, Japan. kanq@takii.kmu.ac.jp
authors.forename: Yasuhide
authors.initials: Y
authors.lastname: Kitazawa
authors.forename: Fukuki
authors.initials: F
authors.lastname: Saito
authors.forename: Shosaku
authors.initials: S
authors.lastname: Nomura
authors.forename: Kazuyoshi
authors.initials: K
authors.lastname: Ishii
authors.forename: Eiji
authors.initials: E
authors.lastname: Kadota
mesh_terms: D000328:Adult; D001853:Bone Marrow / Q000473:pathology; D007446:Chromosome Inversion; D016572:Cyclosporine / Q000097:blood; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008099:Liver / Q000473:pathology; D017114:Liver Failure, Acute / Q000209:etiology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000097:blood / Q000209:etiology* / Q000235:genetics / Q000473:pathology / Q000628:therapy; D008264:Macrophages / Q000473:pathology; D009102:Multiple Organ Failure / Q000209:etiology; D013156:Splenectomy
chemical_list: D016572:Cyclosporine