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目的 探讨发生于肺实质的多中心性巨淋巴结增生(Castleman病)的临床及病理特点.方法 回顾性总结了2008年7月至2013年4月北京协和医院住院及会诊、且经外科肺活检病理诊断的6例发生于肺实质的多中心性巨淋巴结增生患者的临床、影像及病理资料,并复习相关文献.活检标本经4％中性甲醛固定,常规石蜡切片,HE及免疫组织化学染色,3例行B和T淋巴细胞基因重排检测.结果 6例均为女性,年龄31 ～ 68岁,中位年龄为49.5岁.临床表现主要为发热(4例)、咳嗽(3例)及淋巴结肿大(6例)等.实验室检查:ESR为38 ～ 120 mm/1 h(5例),C反应蛋白为23.2 ～1 610 mg/L(4例),丙种球蛋白为27.3％ ～31.5％(2例).胸部CT显示沿淋巴管分布的多发性肺实变结节、磨玻璃影和小叶间隔增宽,伴纵隔多发性淋巴结肿大.病理检查5例为浆细胞型,1例为透明血管型,6例病变区均可见残存的肺泡结构,提示其并非肺内淋巴结的巨淋巴结增生.浆细胞型主要表现为肺间质内大量成熟的浆细胞浸润;透明血管型则以生发中心萎缩和滤泡外套区淋巴细胞呈同心层状排列为特点.B和T淋巴细胞基因重排检测均为阴性.随访2 ～ 60个月(平均31个月),2例浆细胞型行CHOP方案(环磷酰胺+多柔比星+长春新碱+泼尼松)化疗,其余3例未进行任何治疗,病情平稳存活;1例透明血管型行CHOP方案化疗,曾有一过性好转,但于诊断后7个月死于疾病恶化.结论 胸部的巨淋巴结增生常发生于纵隔及肺门或肺内淋巴结,发生于肺实质的巨淋巴结增生罕见,常见于中老年女性,临床表现有呼吸系统症状,并常伴有全身症状,如发热、ESR增快和高丙种球蛋白血症等,胸部CT常表现为沿淋巴管分布的肺内多发性结节及磨玻璃影,常伴纵隔淋巴结肿大.病理组织学改变与淋巴结巨淋巴结增生组织形态特点相似,不同于淋巴细胞性间质性肺炎,免疫组织化学及基因重排检测有助于与其他肺部淋巴组织增生性疾病鉴别.

Objective To observe the clinicopathological features of pulmonary parenchymal involvement of muhicentric Castleman's disease(MCD).Methods 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.Results These 6 patients (all females) aged 31-68 years,with a median of 49.5years.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.Conclusions 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.