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MR Vol.14 No.1 indexに戻る
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MODERN RHEUMATOLOGY
Vol.14 No.1 |
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A case of Weber?Christian disease associated
with myelodysplastic syndrome |
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| Nobumasa Hojo1, Hitoshi Hasegawa1 , Kikue Iwamasa1,
Satoko Hojo2 and Shigeru Fujita1 |
(1) First Department of Internal Medicine,
Ehime University School of Medicine, Shigenobu, Onsen-gun, Ehime,
791-0295, Japan
(2) Daiichi Radiology Hospital, Imabari, Japan |
Received: 19 January 2003 Accepted: 19 June 2003 |
| Abstract |
| Abstract We report the case of a 73-year-old
man with myelodysplastic syndrome (MDS) who developed Weber?Christian
disease (WCD). Bone marrow aspirates showed refractory anemia with
abnormal karyotypes such as trisomy 8, trisomy 8 and 14, and trisomy
8, 9, and 14. The patient had intermittent fever associated with
multiple tender erythematous nodules on the skin. A biopsy sample
taken from a nodule revealed focal subcutaneous infiltration of neutrophils
and necrotizing fat tissue. We diagnosed the patient as having lobular
panniculitis associated with myelodysplastic syndrome. The serum
levels of soluble interleukin-2 (IL-2) receptor, interferon-, IL-1-,
IL-6 and tumor necrosis factor- were elevated in the active state
but returned to normal after prednisolone therapy. This finding appears
to implicate a T-cell immune response in the pathogenesis of Weber?Christian
disease. |
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| Key words |
| Key words Cytokines - Lobular panniculitis -
Myelodysplastic syndrome (MDS) - Trisomy 8 - Weber?Christian disease
(WCD) |
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