Document Detail


Adult-onset Still's disease complicated with hemophagocytic syndrome.
MedLine Citation:
PMID:  20123591     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Horng-Ming Yeh; Ming-Fei Liu; Kuo-Kuan Chang; Shian-Min Liu; Chang-Hung Chen
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
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:
Created Date:  2010-02-03     Completed Date:  2010-04-14     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9214933     Medline TA:  J Formos Med Assoc     Country:  China (Republic : 1949- )    
Other Details:
Languages:  eng     Pagination:  85-8     Citation Subset:  IM    
Affiliation:
Department of Internal Medicine, Tainan Municipal Hospital, Tainan, Taiwan.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:
Azathioprine / 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:
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


Previous Document:  Aortitis With Severe Aortic Regurgitation in Behcet's Disease: A Case Report.
Next Document:  Erratum.