| Adult-onset Still's disease complicated with hemophagocytic syndrome. | |
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MedLine Citation:
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PMID: 20123591 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Hemophagocytic syndrome (HS) that occurs in the course of adult-onset Stills disease (AOSD) has been reported only rarely in the literature. HS and AOSD share overlapping clinical and laboratory features, therefore, it is difficult to recognize HS as a complication of AOSD. Here, we report the case of a 46-year old woman with classical features of AOSD. Severe pancytopenia and jaundice associated with extreme hyperferritinemia occurred during high-dose steroid treatment. Bone marrow biopsy showed typical pathological features of hemophagocytosis, which confirmed the coexistence of HS with AOSD. The patient was treated with methylprednisolone pulse therapy of 500 mg/day for 3 days, as recommended in cases of HS complicating AOSD, and her condition improved gradually. During the disease course, extensive studies could not identify any viral infection or other known underlying etiology for the reactive hemophagocytosis. Currently, the patient is in remission on low-dose prednisolone and azathioprine. |
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Authors:
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Horng-Ming Yeh; Ming-Fei Liu; Kuo-Kuan Chang; Shian-Min Liu; Chang-Hung Chen |
Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Journal of the Formosan Medical Association = Taiwan yi zhi Volume: 109 ISSN: 0929-6646 ISO Abbreviation: J. Formos. Med. Assoc. Publication Date: 2010 Jan |
Date Detail:
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Created Date: 2010-02-03 Completed Date: 2010-04-14 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9214933 Medline TA: J Formos Med Assoc Country: China (Republic : 1949- ) |
Other Details:
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Languages: eng Pagination: 85-8 Citation Subset: IM |
Affiliation:
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Department of Internal Medicine, Tainan Municipal Hospital, Tainan, Taiwan. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Azathioprine
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therapeutic use Bone Marrow / pathology Drug Therapy, Combination Female Fever / etiology Glucocorticoids / administration & dosage Humans Immunosuppressive Agents / therapeutic use Lymphohistiocytosis, Hemophagocytic / complications*, pathology Methylprednisolone / administration & dosage Middle Aged Pulse Therapy, Drug Still's Disease, Adult-Onset / complications*, drug therapy, pathology Treatment Outcome |
| Chemical | |
Reg. No./Substance:
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0/Glucocorticoids; 0/Immunosuppressive Agents; 446-86-6/Azathioprine; 83-43-2/Methylprednisolone |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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