title: Sequential monitoring of serum IL-6, TNF-α, and IFN-γ levels in a CAEBV patient treated by plasma exchange and immunochemotherapy.
abstract: We report the case of a female patient with chronic active Epstein-Barr virus infection (CAEBV) accompanied by hemophagocytic syndrome (HPS). On admission, she presented with severe liver dysfunction and disseminated intravascular coagulation with elevation of serum IL-6, TNF-α, and IFN-γ levels. Plasma exchange (PE) followed by immunochemotherapy with prednisolone, cyclosporine A, and VP16 was performed. PE decreased serum cytokine levels dramatically and improved liver function. Following immunochemotherapy, CAEBV became inactive. Four months after discharge, however, CAEBV relapsed with HPS, and serum cytokine levels were extremely elevated again. There was no response to immunochemotherapy, and the patient died 1 day after admission. We examined the cytokines in five additional untreated-CAEBV patients and determined that they were elevated above the normal level in all patients. These results suggest that inflammatory cytokines may have roles in the development of CAEBV, and that their depletion can be an effective treatment for this disease.
authors.affiliation: Department of Hematology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyoku, Tokyo 113-8519, Japan. ara.hema@tmd.ac.jp
authors.forename: Ayako
authors.initials: A
authors.lastname: Arai
authors.forename: Ayako
authors.initials: A
authors.lastname: Nogami
authors.forename: Ken-Ichi
authors.initials: K
authors.lastname: Imadome
authors.forename: Morito
authors.initials: M
authors.lastname: Kurata
authors.forename: Naomi
authors.initials: N
authors.lastname: Murakami
authors.forename: Shigeyoshi
authors.initials: S
authors.lastname: Fujiwara
authors.forename: Osamu
authors.initials: O
authors.lastname: Miura
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage; D002908:Chronic Disease; D016572:Cyclosporine / Q000008:administration & dosage; D004211:Disseminated Intravascular Coagulation / Q000097:blood / Q000150:complications / Q000628:therapy; D020031:Epstein-Barr Virus Infections / Q000097:blood* / Q000150:complications / Q000628:therapy*; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D004854:Herpesvirus 4, Human; D006801:Humans; D007371:Interferon-gamma / Q000097:blood*; D015850:Interleukin-6 / Q000097:blood*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000097:blood / Q000150:complications / Q000628:therapy; D008875:Middle Aged; D010951:Plasma Exchange; D011239:Prednisolone / Q000008:administration & dosage; D014409:Tumor Necrosis Factor-alpha / Q000097:blood*
chemical_list: C573415:IFNG protein, human; C508600:IL6 protein, human; D015850:Interleukin-6; D014409:Tumor Necrosis Factor-alpha; D005047:Etoposide; D007371:Interferon-gamma; D016572:Cyclosporine; D011239:Prednisolone