Adult-onset Still's Disease Complicated With Hemophagocytic Syndrome
Hemophagocytic syndrome (HS) that occurs in the course of adult-onset Still's 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...
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| Format: | Article |
| Language: | English |
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Elsevier
2010-01-01
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| Series: | Journal of the Formosan Medical Association |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S0929664610600265 |
| _version_ | 1818056506651705344 |
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| author | Horng-Ming Yeh Ming-Fei Liu Kuo-Kuan Chang Shian-Min Liu Chang-Hung Chen |
| author_facet | Horng-Ming Yeh Ming-Fei Liu Kuo-Kuan Chang Shian-Min Liu Chang-Hung Chen |
| author_sort | Horng-Ming Yeh |
| collection | DOAJ |
| description | Hemophagocytic syndrome (HS) that occurs in the course of adult-onset Still's 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. |
| first_indexed | 2024-12-10T12:29:56Z |
| format | Article |
| id | doaj.art-a971cf16284a451699c254433794afa2 |
| institution | Directory Open Access Journal |
| issn | 0929-6646 |
| language | English |
| last_indexed | 2024-12-10T12:29:56Z |
| publishDate | 2010-01-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Journal of the Formosan Medical Association |
| spelling | doaj.art-a971cf16284a451699c254433794afa22022-12-22T01:48:50ZengElsevierJournal of the Formosan Medical Association0929-66462010-01-011091858810.1016/S0929-6646(10)60026-5Adult-onset Still's Disease Complicated With Hemophagocytic SyndromeHorng-Ming Yeh0Ming-Fei Liu1Kuo-Kuan Chang2Shian-Min Liu3Chang-Hung Chen4Department of Internal Medicine, Tainan Municipal Hospital, Tainan, TaiwanDepartment of Internal Medicine, National Cheng Kung University, Tainan, TaiwanDepartment of Internal Medicine, Tainan Municipal Hospital, Tainan, TaiwanDepartment of Pathology, Tainan Municipal Hospital, Tainan, TaiwanDepartment of Internal Medicine, Tainan Municipal Hospital, Tainan, TaiwanHemophagocytic syndrome (HS) that occurs in the course of adult-onset Still's 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.http://www.sciencedirect.com/science/article/pii/S0929664610600265adult-onset Still's diseasehemophagocytic syndromesjaundice |
| spellingShingle | Horng-Ming Yeh Ming-Fei Liu Kuo-Kuan Chang Shian-Min Liu Chang-Hung Chen Adult-onset Still's Disease Complicated With Hemophagocytic Syndrome Journal of the Formosan Medical Association adult-onset Still's disease hemophagocytic syndromes jaundice |
| title | Adult-onset Still's Disease Complicated With Hemophagocytic Syndrome |
| title_full | Adult-onset Still's Disease Complicated With Hemophagocytic Syndrome |
| title_fullStr | Adult-onset Still's Disease Complicated With Hemophagocytic Syndrome |
| title_full_unstemmed | Adult-onset Still's Disease Complicated With Hemophagocytic Syndrome |
| title_short | Adult-onset Still's Disease Complicated With Hemophagocytic Syndrome |
| title_sort | adult onset still s disease complicated with hemophagocytic syndrome |
| topic | adult-onset Still's disease hemophagocytic syndromes jaundice |
| url | http://www.sciencedirect.com/science/article/pii/S0929664610600265 |
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