| Not all hemophagocytes are created equally: appreciating the heterogeneity of the hemophagocytic syndromes. | |
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MedLine Citation:
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PMID: 22089101 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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PURPOSE OF REVIEW: The deadly macrophage activation syndrome (MAS) constitutes one of the few rheumatologic emergencies. MAS is part of a larger group of diseases referred to as hemophagocytic syndromes that are seen in infections, malignancies, or genetic immunodeficiencies. Because of the clinical similarity of these diseases, many clinicians are tempted to approach them all similarly, both in diagnostic criteria and treatment paradigms. New work in the field suggests that not all hemophagocytic syndromes are equal. We will review the latest literature from both human and murine models related to the diagnosis, etiology, and treatment of hemophagocytic syndromes including MAS. RECENT FINDINGS: More specific diagnostic criteria for the different hemophagocytic syndromes are being developed. Animal models suggest at least two different mechanisms by which hemophagocytic syndromes arise: enhanced antigen presentation and excessive Toll-like receptor signaling. Work in humans suggests different cytokine profiles, and different treatment strategies for the variety of hemophagocytic syndromes. SUMMARY: The recent studies reviewed in this article suggest that despite clinical similarities the different hemophagocytic syndromes are indeed likely heterogeneous. Diagnostic criteria and treatment strategies tailored to the underlying disease or genetic context are needed and will hopefully be addressed by future work in this field. |
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Authors:
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Scott W Canna; Edward M Behrens |
Publication Detail:
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Type: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review |
Journal Detail:
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Title: Current opinion in rheumatology Volume: 24 ISSN: 1531-6963 ISO Abbreviation: Curr Opin Rheumatol Publication Date: 2012 Jan |
Date Detail:
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Created Date: 2011-12-05 Completed Date: 2012-03-27 Revised Date: 2013-05-23 |
Medline Journal Info:
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Nlm Unique ID: 9000851 Medline TA: Curr Opin Rheumatol Country: United States |
Other Details:
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Languages: eng Pagination: 113-8 Citation Subset: IM |
Affiliation:
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The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Animals Humans Lymphohistiocytosis, Hemophagocytic* / diagnosis, etiology, therapy Macrophage Activation Syndrome / diagnosis, etiology, therapy Mice |
| Grant Support | |
ID/Acronym/Agency:
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K08 AI079396/AI/NIAID NIH HHS; K08 AI079396-04/AI/NIAID NIH HHS; K08-AI079396/AI/NIAID NIH HHS; T32-AR007442/AR/NIAMS NIH HHS; //Howard Hughes Medical Institute |
| Comments/Corrections | |
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