[A case of systemic lupus erythematosus with hemophagocytic syndrome and cytophagic histiocytic panniculitis].
[A case of systemic lupus erythematosus with hemophagocytic syndrome and cytophagic histiocytic panniculitis].
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系统性红斑狼疮合并噬血细胞综合征并噬细胞性组织细胞性脂膜炎1例[J].
DOI：
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发表时间：
2001
期刊：
影响因子：
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通讯作者：
E. Tanabe
中科院分区：
文献类型：
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作者：
Y. Mori;T. Sugiyama;R. Chiba;T. Sugimoto;M. Furukawa;M. Sueishi;E. Tanabe
一名23岁男性因高烧、多关节痛、蝶形皮疹和胸痛入院，经抗核抗体和抗DNA抗体阳性诊断为系统性红斑狼疮（SLE）。每日给予泼尼松龙60mg治疗，但随着剂量减少，白细胞计数和血小板计数下降，并出现发热、多关节痛、补体减少、铁蛋白升高、肝脾肿大和肝功能障碍。骨髓标本显示组织细胞吞噬血细胞，诊断为活动性系统性红斑狼疮导致的噬血细胞综合征（HPS）。甲泼尼龙冲击治疗暂时有效，减少类固醇后HPS复发，加用环孢素。暂时缓解后，脸部突然出现明显的大面积肿胀。面部皮肤活检显示脂肪细胞坏死和组织细胞噬血。据此，他被诊断为HPS引起的脂膜炎，并通过静脉环磷酰胺冲击疗法和大剂量丙种球蛋白成功治疗。最近报道了几例由 SLE 引起的 HPS 病例，但这是由 SLE 引起的噬细胞组织细胞性脂膜炎 (CHP) 的罕见病例。
A 23-year-old man, admitted because of high fever, polyarthralgia, butterfly rash and chest pain, was diagnosed as systemic lupus erythematosus (SLE) from the findings of positive antinuclear antibody and anti-DNA antibody. He was treated with 60 mg prednisolone daily, but as reducing the dose, white blood cell counts and platelet counts were decreased and fever, polyarthralgia, decrease of complements, increase of ferritin, hepato-splenomegaly and liver dysfunction were observed. Bone marrow specimen revealed phagocytosis of blood cells by histiocytes and he was diagnosed as hemophagocytic syndrome(HPS) due to active SLE. Methylprednisolone pulse therapy was effective temporarily, HPS recurred while reducing steroid, and cyclosporin was added. After a temporary remission, marked extensive swelling in the face appeared suddenly. Facial skin biopsy showed necrosis of fat cells and hemophagocytosis by histiocytes. Accordingly, he was diagnosed as panniculitis due to HPS and was treated successfully with intravenous cyclophosphamide pulse therapy and high dose of gammaglobulin. Several cases of HPS due to SLE have been reported recently, but this is a rare case of cytophagic histiocytic panniculitis (CHP) due to SLE.