Sequential monitoring of serum IL-6, TNF-α, and IFN-γ levels in a CAEBV patient treated by plasma exchange and immunochemotherapy

• Published in: International journal of hematology Vol. 96; no. 5; pp. 669 - 673
• Main Authors: Arai, Ayako, Nogami, Ayako, Imadome, Ken-Ichi, Kurata, Morito, Murakami, Naomi, Fujiwara, Shigeyoshi, Miura, Osamu
• Format: Journal Article
• Language: English
• Published: Japan Springer Japan 01.11.2012; Springer
• Subjects: Antineoplastic Combined Chemotherapy Protocols - administration & dosage; Biological and medical sciences; Case Report; Chronic Disease; Cyclosporine - administration & dosage; Disseminated Intravascular Coagulation - blood; Disseminated Intravascular Coagulation - complications; Disseminated Intravascular Coagulation - therapy; Epstein-Barr Virus Infections - blood; Epstein-Barr Virus Infections - complications; Epstein-Barr Virus Infections - therapy; Etoposide - administration & dosage; Female; Hematologic and hematopoietic diseases; Hematology; Herpesvirus 4, Human; Humans; Infectious diseases; Interferon-gamma - blood; Interleukin-6 - blood; Lymphohistiocytosis, Hemophagocytic - blood; Lymphohistiocytosis, Hemophagocytic - complications; Lymphohistiocytosis, Hemophagocytic - therapy; Medical sciences; Medicine; Medicine & Public Health; Middle Aged; Oncology; Other diseases. Hematologic involvement in other diseases; Plasma Exchange; Prednisolone - administration & dosage; Tumor Necrosis Factor-alpha - blood; Viral diseases; Hemophagocytic syndrome; IFN-γ; TNF-α; Chronic active Epstein–Barr virus infection; IL-6; Immunochemotherapy; Alpha interferon; Hemopathy; Epstein Barr virus; Interleukin 6; Plasma exchange; Sequential; Immunological investigation; Chronic active Epstein―Barr virus infection; Tumor necrosis factor α; Gammaherpesvirinae; Human; Hematology; Herpesviridae; Cytokine; Histiocytosis; Virus; Infection; Chemotherapy; Chronic; Treatment; Surveillance; Viral disease; Gamma interferon
• ISSN: 0925-5710; 1865-3774
• DOI: 10.1007/s12185-012-1170-2

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.