An Unusual Case of Systemic Lupus Erythematosus and Hemophagocytic Syndrome

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...

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Main Authors: Saika Sharmeen, Nazia Hussain
Format: Article
Language:English
Published: Wiley 2016-01-01
Series:Case Reports in Rheumatology
Online Access:http://dx.doi.org/10.1155/2016/8957690
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author Saika Sharmeen
Nazia Hussain
author_facet Saika Sharmeen
Nazia Hussain
author_sort Saika Sharmeen
collection DOAJ
description 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.
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spelling doaj-art-2e7c252243e54aafae9f47ddd68751652025-02-03T01:22:50ZengWileyCase Reports in Rheumatology2090-68892090-68972016-01-01201610.1155/2016/89576908957690An Unusual Case of Systemic Lupus Erythematosus and Hemophagocytic SyndromeSaika Sharmeen0Nazia Hussain1Department of Medicine, St. Luke’s-Roosevelt Hospital Center, New York, NY 10025, USADepartment of Medicine, Icahn School of Medicine at Mount Sinai, New York, NY 10025, USAHemophagocytic 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.http://dx.doi.org/10.1155/2016/8957690
spellingShingle Saika Sharmeen
Nazia Hussain
An Unusual Case of Systemic Lupus Erythematosus and Hemophagocytic Syndrome
Case Reports in Rheumatology
title An Unusual Case of Systemic Lupus Erythematosus and Hemophagocytic Syndrome
title_full An Unusual Case of Systemic Lupus Erythematosus and Hemophagocytic Syndrome
title_fullStr An Unusual Case of Systemic Lupus Erythematosus and Hemophagocytic Syndrome
title_full_unstemmed An Unusual Case of Systemic Lupus Erythematosus and Hemophagocytic Syndrome
title_short An Unusual Case of Systemic Lupus Erythematosus and Hemophagocytic Syndrome
title_sort unusual case of systemic lupus erythematosus and hemophagocytic syndrome
url http://dx.doi.org/10.1155/2016/8957690
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