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一例系统性红斑狼疮患者胸腺切除术后并发纯红细胞再生障碍性贫血和特发性门静脉高压症。

A case of systemic lupus erythematosus complicated by pure red cell aplasia and idiopathic portal hypertension after thymectomy.

作者信息

Iwadate Haruyo, Kobayashi Hiroko, Shio Kiori, Noguchi Etsuko, Watanabe Kenya, Sasajima Tomomi, Sekine Hideharu, Watanabe Hiroshi, Ohira Hiromasa, Obara Katsutoshi, Sato Yukio

机构信息

Department of Internal Medicine II, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima, 960-1295, Japan.

出版信息

Mod Rheumatol. 2006;16(2):109-12. doi: 10.1007/s10165-006-0465-7.

Abstract

We describe a 49-year-old woman who presented in 2002 with pure red cell aplasia (PRCA), systemic lupus erythematosus (SLE), and idiopathic portal hypertension (IPH) that developed following a thymectomy. She underwent a thymectomy at 40 years of age to treat myasthenia gravis. PRCA developed 3 years after the thymectomy and she was successfully treated with cyclosporin. Systemic lupus erythematosus and IPH were diagnosed 6 years later. We conclude that immunological dysfunction resulting from the thymectomy contributed significantly to the subsequent development of PRCA, SLE, and IPH in this patient. This is the first report to describe this extremely rare occurrence.

摘要

我们描述了一位49岁女性,她于2002年出现纯红细胞再生障碍性贫血(PRCA)、系统性红斑狼疮(SLE)和胸腺切除术后发生的特发性门静脉高压(IPH)。她40岁时接受胸腺切除术以治疗重症肌无力。PRCA在胸腺切除术后3年出现,她接受环孢素治疗成功。6年后诊断出系统性红斑狼疮和IPH。我们得出结论,胸腺切除术导致的免疫功能障碍在很大程度上促成了该患者随后发生的PRCA、SLE和IPH。这是描述这种极其罕见情况的首例报告。

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