An Unusual Case of Systemic Lupus Erythematosus and Hemophagocytic Syndrome. (14th February 2016)

• Record Type: Journal Article
• Authors: Sharmeen, Saika; Hussain, Nazia
• Other Names: Ikuyama Shoichiro Academic Editor.
• Is Part Of: Case reports in rheumatology. Volume 2016(2016)
• Publication Date: 2016-02-14
• Subjects: Rheumatology -- Periodicals; Rheumatology -- Case studies -- Periodicals; Rheumatic Diseases; Connective Tissue Diseases; Rheumatology; Periodicals; Electronic journals; Case studies; Periodicals; 616.723
• DOI: 10.1155/2016/8957690
• Languages: English
• ISSNs: 2090-6889

Abstract

Abstract : Hemophagocytic syndrome (HS) or hemophagocytic lymphohistiocytosis (HLH) is an immune mediated phenomenon that can occur in the setting of an autoimmune disease, chronic immunosuppression, malignancy, or infection. It has been more commonly described in the pediatric population and less commonly in adults. We describe a case of a 52-year-old male who presented with a rash. He simultaneously met the Systemic Lupus International Collaborating Clinics (SLICC) criteria for the diagnosis of systemic lupus erythematosus (SLE) and the diagnostic criteria of HS as described in the hemophagocytic lymphohistiocytosis (HLH) 2004 trial. The bone marrow on autopsy showed the presence of abundant hemosiderophages with focal hemophagocytosis. SLE-associated HS might be underdiagnosed due to the overlap in clinical findings. This case represents the importance of prompt diagnosis and treatment of such a potentially fatal clinical syndrome.