![]() Treatment outcome was at 77% survival, while 23% had fatal outcome.Ĭonclusion: The findings highlight the importance of early diagnostic work up and treatment intervention. The general treatment approach was in general dual, consisting of antiviral treatment and specific immunomodulating HLH treatment approaches. Forty patients (54%) received some form of immunomodulating treatment prior to HLH development. Sixty-six patients (88%) had one or more comorbid conditions and 22 patients (30%) had inflammatory bowel disease (IBD), the most frequent comorbidity. Results: A total of 74 patients (age > 18 years) with CMV-related HLH were identified, 29 men, 42 women, and three patients with unspecified gender. All articles were assessed on a predetermined set of inclusion criteria. Methods: We report a recent case of CMV-induced HLH, followed by a systematic review of described cases of this rare disease entity, through a structured search in the medical database PubMed. Cytomegalovirus (CMV) is a virus in the herpes family known to cause HLH in rare cases. Viral infections are a well-known cause of secondary HLH. Primary HLH is caused by genetic mutations, while secondary HLH is triggered by external factors. It is driven by an overactivation of the immune system and causes inflammatory tissue damage potentially leading to organ failure and death. 4Department of Pathology, Haukeland University Hospital, Bergen, Norwayīackground: Hemophagocytic lymphohistiocytosis (HLH) is an immune mediated life-threatening condition.3Department of Medical Science, Faculty of Medicine, University of Bergen, Bergen, Norway. ![]() 2Department of Medicine, Haukeland University Hospital, Bergen, Norway.1Department of Clinical Science, Faculty of Medicine, University of Bergen, Bergen, Norway.Linn Åsholt Rolsdorph 1, Knut Anders Mosevoll 1,2, Lars Helgeland 3,4 and Håkon Reikvam 1,2 *
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