[噬血细胞综合征作为产后系统性红斑狼疮患者的主要表现]
[Hemophagocytic syndrome as primary manifestation in a patient with systemic lupus erythematosus after parturition].
作者信息
Yoshida Shuzo, Takeuchi Tohru, Itami Yasuo, Hata Kenichiro, Watanabe Koko, Shoda Takeshi, Kotani Takuya, Makino Shigeki, Hanafusa Toshiaki
机构信息
First Department of Internal Medicine, Osaka Medical College, Takatsuki, Osaka.
出版信息
Nihon Rinsho Meneki Gakkai Kaishi. 2009 Feb;32(1):66-70. doi: 10.2177/jsci.32.66.
A 33-year-old woman presented with fever, malar rash, lymphadenopathy and pancytopenia 3 weeks after parturition. Serum C3 level was decreased and antinuclear antibody, anti-dsDNA antibody and anti-SS-A antibody were positive. Serum ferritin level was elevated (3454 ng/ml), and bone marrow aspirate revealed hemophagocytosis. She was diagnosed having systemic lupus erythematosus (SLE) associated with hemophagocytic syndrome (HPS). Oral prednisolone (55 mg/day) was initiated. Clinical manifestations and pancytopenia were improved and serum ferritin level was decreased. However, elevated anti-dsDNA antibody titer and reduced C3 level continued for a month after steroid therapy. The additional therapy of tacrolimus (3 mg/day) improved clinical and laboratory findings. This is a rare SLE case associated with HPS as primary manifestation after parturition.
一名33岁女性在产后3周出现发热、颧部皮疹、淋巴结病和全血细胞减少。血清C3水平降低，抗核抗体、抗双链DNA抗体和抗SS - A抗体均为阳性。血清铁蛋白水平升高（3454 ng/ml），骨髓穿刺显示噬血细胞现象。她被诊断为患有系统性红斑狼疮（SLE）伴噬血细胞综合征（HPS）。开始口服泼尼松龙（55 mg/天）。临床表现和全血细胞减少得到改善，血清铁蛋白水平降低。然而，类固醇治疗后抗双链DNA抗体滴度升高和C3水平降低持续了一个月。他克莫司（3 mg/天）的附加治疗改善了临床和实验室检查结果。这是一例产后以HPS为主要表现的罕见SLE病例。
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