title: Hemophagocytic lymphohistiocytosis in a patient with Sjögren's syndrome: case report and review.
abstract: Hemophagocytic lymphohistiocytosis (HLH) is a very rare syndrome with a mortality up to 95% of cases if not treated. It is characterised by an excessive activation of the immune system that leads to a disproportionate and destructive inflammatory response. The high mortality rates are in part due to a delay in the diagnosis, and therefore clinicians must maintain a high index of suspicion. When the treatment is started early, the survival rate reaches around 55% of cases. HLH usually presents with persistent fever, pancytopenia, and organomegaly and is associated with very high levels of serum ferritin. In this manuscript, we present the case of a patient with primary Sjögren's syndrome who developed HLH after an acute infection by Cytomegalovirus. We will describe and discuss the pathogenesis, differential diagnosis and a pragmatic approach to the treatment for this critically important and, when diagnosed early, potentially curable syndrome.
authors.affiliation: Department of Rheumatology, Hospital General Universitario Gregorio Marañón, 46, Dr. Esquerdo, 28007, Madrid, Spain. leticia@garciam.net.
authors.forename: L
authors.identifier: http://orcid.org/0000-0001-9518-2887
authors.initials: L
authors.lastname: García-Montoya
authors.affiliation: Department of Rheumatology, Hospital General Universitario Gregorio Marañón, 46, Dr. Esquerdo, 28007, Madrid, Spain.
authors.forename: C N
authors.initials: CN
authors.lastname: Sáenz-Tenorio
authors.affiliation: Department of Rheumatology, Hospital General Universitario Gregorio Marañón, 46, Dr. Esquerdo, 28007, Madrid, Spain.
authors.forename: I
authors.initials: I
authors.lastname: Janta
authors.affiliation: Department of Rheumatology, Hospital General Universitario Gregorio Marañón, 46, Dr. Esquerdo, 28007, Madrid, Spain.
authors.forename: J
authors.initials: J
authors.lastname: Menárguez
authors.affiliation: Department of Rheumatology, Hospital General Universitario Gregorio Marañón, 46, Dr. Esquerdo, 28007, Madrid, Spain.
authors.forename: F J
authors.initials: FJ
authors.lastname: López-Longo
authors.affiliation: Department of Rheumatology, Hospital General Universitario Gregorio Marañón, 46, Dr. Esquerdo, 28007, Madrid, Spain.
authors.forename: I
authors.initials: I
authors.lastname: Monteagudo
authors.affiliation: Department of Rheumatology, Hospital General Universitario Gregorio Marañón, 46, Dr. Esquerdo, 28007, Madrid, Spain.
authors.forename: E
authors.initials: E
authors.lastname: Naredo
mesh_terms: D000368:Aged; D003586:Cytomegalovirus Infections / Q000150:complications*; D005260:Female; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000209:etiology*; D012859:Sjogren's Syndrome / Q000150:complications*
keywords: CMV; Cytomegalovirus; HLH; Hemophagocytic lymphohistiocytosis; Sjögren's syndrome