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结节病17年随访期间发生的混合型多中心Castleman病。

Mixed-type multicentric Castleman's disease developing during a 17-year follow-up of sarcoidosis.

作者信息

Awano Nobuyasu, Inomata Minoru, Kondoh Keisuke, Satake Kohta, Kamiya Hiroyuki, Moriya Atsuko, Ando Tsunehiro, Kumasaka Toshio, Takemura Tamiko, Takeuchi Kengo, Ikushima Soichiro

机构信息

Department of Respiratory Medicine, Japanese Red Cross Medical Center, Japan.

出版信息

Intern Med. 2012;51(21):3061-6. doi: 10.2169/internalmedicine.51.8120. Epub 2012 Nov 1.

Abstract

Multicentric Castleman's Disease (MCD) is a systemic disease characterized by generalized lymphadenopathy and the proliferation of plasma cells. The development of MCD in a patient with preexisting sarcoidosis has not been previously reported. We herein describe a case of MCD developing in a 78-year-old woman with a 17-year history of sarcoidosis. The patient's serum interleukin-6 (IL-6) levels were only slightly elevated; however, the IL-6 levels in the fluid of both pleural effusion and ascites were markedly elevated. The administration of steroid-pulse therapy and prednisolone was ineffective in treating the MCD, although treatment with tocilizumab proved highly effective.

摘要

多中心性Castleman病(MCD)是一种以全身淋巴结肿大和浆细胞增殖为特征的系统性疾病。既往未曾有过在已有结节病的患者中发生MCD的报道。我们在此描述一例78岁、有17年结节病病史的女性发生MCD的病例。该患者的血清白细胞介素-6(IL-6)水平仅略有升高;然而,胸腔积液和腹水液中的IL-6水平均显著升高。尽管托珠单抗治疗证明非常有效,但类固醇脉冲疗法和泼尼松龙治疗对MCD无效。

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