Tian Xinlun, Ge Li, Feng Rui'e, Liu Tao, Huang Hui, Xu Zuojun, Xu Wenbing, Liu Hongrui
Department of Respiration, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing 100730, China.
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Zhonghua Jie He He Hu Xi Za Zhi. 2014 May;37(5):337-42.
To observe the clinicopathological features of pulmonary parenchymal involvement of multicentric Castleman's disease(MCD).
Retrospective analysis was carried out for 6 patients of MCD with pulmonary parenchymal involvement who had been admitted to Peking Union Medical College Hospital from July 2008 to March 2013. Relevant literatures were reviewed. The diagnosis was established by surgical lung biopsy and all specimens were fixed in neutral formalin and embedded in paraffin. Sections were cut for HE and immunohistochemical stain. B cell and T cell gene rearrangement were tested in 3 cases.
These 6 patients (all females) aged 31-68 years, with a median of 49.5 years. The presenting symptoms were fever (4/6), cough (3/6), and lymphadenopathy (6/6). Laboratory study showed elevated ESR (5/6) and CRP (4/6), and hypergammaglobulinaemia (2/6). Chest CT showed multiple nodules with perilymphatic distribution and ground-glass opacity (GGO). Pathologically, there were 5 cases of plasma cell type and 1 case of hyaline vascular type. The plasma cell variant showed dense mature plasma cell infiltration in pulmonary interstitium. The hyaline vascular variant was characterized by the presence of regressed germinal centers and broad concentric mantle zones. The gene arrangement tests were all negative. During the follow-up period (range: 2-60 months; mean: 31 months), 2 cases with plasma cell type received CHOP chemotherapy and then remained stable. One case with hyaline vascular type received CHOP chemotherapy but died due to deterioration of the disease.
In the thorax, Castleman's disease usually manifests as hilar and mediastinal lymph node enlargement. Pulmonary parenchymal involvement by MCD is very rare. It is mostly seen in the elderly female, and can manifest with systemic symptoms. Chest CT usually reveals multiple nodules and GGO. It shows similar morphological characteristics to those found in lymph nodes. Immunohistochemistry and gene rearrangement test can help to differentiate it from other pulmonary lymphoproliferative diseases.
观察多中心Castleman病（MCD）肺实质受累的临床病理特征。
对2008年7月至2013年3月在北京协和医院住院的6例MCD肺实质受累患者进行回顾性分析，并复习相关文献。通过手术肺活检确诊，所有标本用中性福尔马林固定，石蜡包埋。切片进行HE和免疫组化染色。对3例患者检测B细胞和T细胞基因重排。
这6例患者均为女性，年龄31 - 68岁，中位年龄49.5岁。主要症状为发热（4/6）、咳嗽（3/6）和淋巴结肿大（6/6）。实验室检查显示血沉升高（5/6）、C反应蛋白升高（4/6）和高球蛋白血症（2/6）。胸部CT显示多发结节呈淋巴管周围分布及磨玻璃影（GGO）。病理上，5例为浆细胞型，1例为透明血管型。浆细胞型表现为肺间质内密集成熟浆细胞浸润。透明血管型的特征为存在退化的生发中心和宽阔的同心性套区。基因重排检测均为阴性。随访期间（范围：2 - 60个月；平均：31个月），2例浆细胞型患者接受CHOP化疗后病情稳定。1例透明血管型患者接受CHOP化疗，但因病情恶化死亡。
在胸部，Castleman病通常表现为肺门和纵隔淋巴结肿大。MCD肺实质受累非常罕见。多见于老年女性，可伴有全身症状。胸部CT通常显示多发结节和GGO。其形态学特征与淋巴结所见相似。免疫组化和基因重排检测有助于将其与其他肺淋巴增殖性疾病鉴别。
