Hemophagocytic lymphohistiocytosis associated with herpes infection

Abstract

G.S. Karpovich1,2, A.E. Shestakov1, E.I. Krasnova1 1Novosibirsk State Medical University, Novosibirsk, Russian Federation 2Children’s City Clinical Hospital No. 3, Novosibirsk, Russian Federation Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal disease affecting multiple organs and systems which is induced by the dysregulation of the immune response due to mutations in the genes controlling cytolytic function. This leads to the abnormal activation of cytotoxic T cells, monocytes/macrophages, and the development of a systemic inflammatory response. In some cases, patients with HLH require emergency therapy in intensive care or resuscitation units. The article highlights the issues of HLH etiopathogenesis, its clinical, diagnostic and treatment aspects. As association was found between secondary HLH and infections caused by the herpesvirus group. The diagnosis of this condition is challenging because of its nonspecific symptoms, especially in early stages of the disease, which leads to an uncertain prognosis. The authors present case reports of two pediatric patients with secondary HLH induced by herpesvirus mixed infection. The clinical cases were characterized by the manifestations of acute dual infection with Epstein-Barr virus and cytomegalovirus, and HLH symptoms that were consistent with the diagnostic criteria of the disease described in the article. For selecting an adequate therapy, it is necessary to conduct high-quality comprehensive diagnostic testing with the involvement of hematologists, rheumatologists. and oncologists. Keywords: hemophagocytic lymphohistiocytosis, infectious diseases, herpes virus, Epstein-Barr virus, cytomegalovirus, hematology, mixed infection. For citation: Karpovich G.S., Shestakov A.E., Krasnova E.I. Hemophagocytic lymphohistiocytosis associated with herpes infection. Russian Journal of Woman and Child Health. 2022;5(3):262–269 (in Russ.). DOI: 10.32364/2618-8430-2022-5-3-262-269.