Abstract
A 59-year-old man, who was treated for schizophrenia and psoriasis vulgaris with risperidone and cyclosporine, presented with high fever and myalgia. Those did not respond to treatment for neuroleptic malignant syndrome for two weeks, and multiple organ dysfunction developed, so he was admitted to our hospital, but died two days later. Autopsy detected the hemophagocytosis, Epstein Barr Virus (EBV)-reactivated cells, and the absence of glomerulonephritis and interstitial tubulitis. We considered that hemophagocytic syndrome (HPS) and myalgia were caused by reactivated EBV and the viremia under immunosuppression, and renal failure was caused by sepsis-like state by cytokine storm of HPS.
Highlights
Hemophagocytic syndrome (HPS) is a rare disorder of the immune system that is synonymous with macrophage activation syndrome [1]
Myalgia is a common symptom of Epstein Barr Virus (EBV) infection, and rhabdomyolysis associated with EBV infection has been reported [10] [11]
In the development of EBV-associated HPS, latent EBV in B cells is reactivated under immunosuppression, proliferates, and infects Natural killer (NK)/T cells through the viremia of EBV
Summary
Hemophagocytic syndrome (HPS) is a rare disorder of the immune system that is synonymous with macrophage activation syndrome [1]. Natural killer (NK)/T cells that, for hereditary or acquired reasons, are unable to lyse target cells, such as infected or malignant cells, proliferate and release a large amount of interferon-γ. This induces the proliferation of macrophages, invasion into the liver, spleen, and lymph nodes, and the release of further inflammatory cytokines including TNF-α [2]. (2016) Acute Kidney Injury with Epstein Barr Virus-Associated Hemophagocytic Syndrome. (EBV) accounts for 43% of viral infections [4]. We report a case of EBV-associated HPS receiving immunosuppressive therapy. We discuss the pathophysiology of acute kidney injury (AKI) and the clinical symptoms preceding HPS
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