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Review
. 2017 Apr;37(4):663-669.
doi: 10.1007/s00296-016-3601-5. Epub 2016 Nov 16.

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

L García-Montoya  1 , C N Sáenz-Tenorio  2 , I Janta  2 , J Menárguez  2 , F J López-Longo  2 , I Monteagudo  2 , E Naredo  2
Affiliations
Review

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

L García-Montoya et al. Rheumatol Int. 2017 Apr.

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.

Keywords: CMV; Cytomegalovirus; HLH; Hemophagocytic lymphohistiocytosis; Sjögren's syndrome.

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Conflict of interest statement

Leticia García-Montoya, Claudia Sáenz-Tenorio, Iustina Janta, Javier Menárguez, Indalecio Monteagudo and Esperanza Naredo declare that they have no conflict of interest. Javier López-Longo reports personal fees from Abbvie, Roche Farma, Bristol-Myers Squibb, Pfizer, UCB, MSD and Actelion, grants from Abbvie and GSK, outside the submitted work.

Figures

Fig. 1
Fig. 1
Evolution of the viral load once the treatment was started. CMV cytomegalovirus
Fig. 2
Fig. 2
Diagnostic criteria based on the 2004 HLH trial [47]
Fig. 3
Fig. 3
Representation of the main aspects for the treatment of HLH syndrome. BM bone marrow, Ig i/v intravenous immunoglobulin
See this image and copyright information in PMC

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