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Case Reports in Rheumatology
Volume 2016, Article ID 8957690, 5 pages
Case Report

An Unusual Case of Systemic Lupus Erythematosus and Hemophagocytic Syndrome

1Department of Medicine, St. Luke’s-Roosevelt Hospital Center, New York, NY 10025, USA
2Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, NY 10025, USA
3Division of Rheumatology, Department of Medicine, St. Luke’s-Roosevelt Hospital Center, New York, NY 10025, USA

Received 2 November 2015; Revised 5 January 2016; Accepted 13 January 2016

Academic Editor: Shoichiro Ikuyama

Copyright © 2016 Saika Sharmeen and Nazia Hussain. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


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.