Hemophagocytic lymphohistiocytosis in a patient with Sjögren’s syndrome: case report and review

• Published in: Rheumatology International 2017-04, Vol.37 (4), p.663-669
• Main Authors: García-Montoya, L., Sáenz-Tenorio, C. N., Janta, I., Menárguez, J., López-Longo, F. J., Monteagudo, I., Naredo, E.
• Format: Article
• Language: English
• Subjects: Aged; Cases with a Message; Cytomegalovirus Infections - complications; Female; Humans; Lymphohistiocytosis, Hemophagocytic - etiology; Medicine; Medicine & Public Health; Rheumatology; Sjogren's Syndrome - complications
• ISSN: 0172-8172; 1437-160X
• DOI: 10.1007/s00296-016-3601-5

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.