Okamoto Masaki, Hoshino Tomoaki, Nakamura Masayuki, Imamura Yohei, Fujimoto Kiminori, Oshima Koichi, Kawayama Tomotaka, Aizawa Hisamichi
Division of Respirology, Neurology, and Rheumatology, Department of Internal Medicine, Kurume University School of Medicine.
Nihon Kokyuki Gakkai Zasshi. 2011 Apr;49(4):266-70.
A 31-year-old man visited our hospital with a persistent cough. Computed tomography (CT) scans of his chest showed a very large mass and multiple nodular lesions in the right lung field, mediastinal and hilar lymph node enlargement and splenomegaly. Laboratory analysis showed polyclonal hyperimmunoglobulinemia and increased levels of serum C-reactive protein (14.05 mg/dl) and interleukin-6 (44.2 pg/ml). The pathological findings of lung specimens obtained using video-assisted thoracoscopy revealed hyperplasia of the lymphoid follicles with germinal centers, plasma cell infiltration which stained positively with either anti-kappa chain or anti-lambda chain antibodies, and fibrosis in the alveolar septum. We made a diagnosis of multicentric Castleman disease based on high levels of serum IL-6, multiple lymph node enlargement and splenomegaly, although this case had histological findings in common not only with Castleman disease but also with inflammatory myofibroblastic tumor. His abnormal chest radiography findings and laboratory data significantly improved 6 months after his first visit, without any treatment. Multicentric Castleman disease showing a very large mass is extremely rare.
一名31岁男性因持续性咳嗽前来我院就诊。其胸部计算机断层扫描(CT)显示右肺野有一个非常大的肿块及多个结节性病变,纵隔和肺门淋巴结肿大以及脾肿大。实验室分析显示多克隆高免疫球蛋白血症,血清C反应蛋白(14.05mg/dl)和白细胞介素-6(44.2pg/ml)水平升高。使用电视辅助胸腔镜获取的肺标本病理结果显示有生发中心的淋巴滤泡增生、浆细胞浸润,抗κ链或抗λ链抗体染色呈阳性,以及肺泡间隔纤维化。尽管该病例的组织学表现不仅与Castleman病有共同之处,还与炎性肌纤维母细胞瘤有共同之处,但基于血清IL-6水平升高、多处淋巴结肿大和脾肿大,我们诊断为多中心Castleman病。首次就诊6个月后,未经任何治疗,他异常的胸部X线检查结果和实验室数据显著改善。表现为非常大肿块的多中心Castleman病极为罕见。