30例不同类型胸部受累Castleman病临床分析

目的总结不同类型胸部受累Castleman病(CD)的临床、影像学、病理及诊治特征,以提高临床医师对CD的认识。方法回顾性分析广州医科大学附属第一医院自2009年6月至2019年5月收治入院的胸部受累CD患者30例,将其分为闭塞性细支气管炎(BO)组、不伴BO的单中心型Castleman病(UCD)组及不伴BO的多中心型Castleman病(MCD)组,分析其临床资料并总结其特征。结果30例患者中,5例(16.7%)纳入BO组,18例(60.0%)纳入不伴BO的UCD组,7例(23.3%)纳入不伴BO的MCD组。不伴BO的MCD组中位年龄明显大于BO组(49.29±5.39)岁对(27.20±3.76)岁,P=0.005]和不伴BO的UCD组(49.29±5.39)岁对(37.17±2.87)岁,P=0.034]。胸部症状在BO组(100%)和不伴BO的MCD组(71.4%)多见,而不伴BO的UCD组无胸部症状。贫血及IgG增高仅在不伴BO的MCD组出现(发生率均为57.1%),红细胞沉降率增快及低氧血症在BO组(发生率分别为40.0%和60.0%)及不伴BO的MCD组(发生率分别为57.1%和28.6%)均可出现。BO组患者的肺功能均表现为极重度混合性通气功能障碍。胸部CT所示肺实质受累率:BO组为100%;不伴BO的MCD组为57.1%,表现为双肺弥漫性病变;不伴BO的UCD组为11.1%,表现为孤立性肺结节。不伴BO的MCD组的淋巴结短径明显小于BO组(1.83±0.51)cm对(4.73±1.63)cm,P=0.006]和不伴BO的UCD组(1.83±0.51)cm对(3.62±0.26)cm,P=0.011]。BO组病理类型均为透明血管型(100%),不伴BO的UCD组88.9%为透明血管型,而不伴BO的MCD组以浆细胞型为主(57.1%)。BO组患者均出现口腔溃疡,在肿物切除术后予免疫调节剂治疗,溃疡症状缓解,但肺部症状仍进行性加重。不伴BO的UCD组主要治疗方式为胸腔镜下肿物切除,不伴BO的MCD组主要治疗方式为化疗、免疫调节剂、靶向治疗等。结论三组患者的年龄、临床症状、实验室检查、肺功能、影像学表现、病理类型、治疗及预后均有所不同,此分类能促进临床医师对本病的认识。

Clinical analysis of 30 cases of Castleman disease with different types of thoracic involvement

Objective To improve the clinical understanding of Castleman disease(CD)with different types of thoracic involvement,including their clinical features,radiological and pathological findings,diagnosis and current treatment strategies.Methods Retrospective analysis of 30 patients diagnosed with CD with thoracic involvement and hospitalized between June 2009 and May 2019 in The First Affiliated Hospital of Guangzhou Medical University was performed.Patients were divided into three groups for subsequent analysis based on the clinical data:CD with bronchiolitis obliterans(BO),unicentric Castleman disease(UCD)without BO,and multicentric Castleman disease(MCD)without BO.Results Among the 30 patients,there were 5(16.7%)patients diagnosed with BO,18(60.0%)patients had UCD without BO and 7(23.3%)patients had MCD without BO.The average age of MCD without BO patients was significantly older than that of BO and UCD without BO patients(49.29±5.39)ys vs(27.20±3.76)ys and(37.17±2.87)ys;P=0.005 and 0.034,respectively)].Pulmonary symptoms were commonly seen in BO group(100%)and MCD without BO group(71.4%).while no pulmonary symptoms were seen in UCD without BO group.Key abnormal laboratory findings were erythrocyte sedimentation rate(ESR)increase(40%in BO group and 57.1%in MCD without BO group)and hypoxia(60%in BO group and 28.6%in MCD without BO group).Other abnormal laboratory findings seen in MCD without BO group included anemia and IgG increase(both 57.1%).Notably,all patients in BO group had extremely severe mixed ventilation dysfunction in the lung function test.CT scan showed lung parenchyma involvement in BO group(100%),in UCD without BO group(11.1%)featured by solitary pulmonary nodule and in MCD without BO group(57.1%)featured by diffuse lesions in bilateral lungs.The size of lymph nodes was significantly smaller in MCD without BO group comparing to that in BO group and UCD without BO groupshort diameter(1.83±0.51)cm vs(4.73±1.63)cm and(3.62±0.26)cm;P=0.006 and 0.011,respectively].All patients(100%)in the BO group had a pathological type of transparent vascular variant while the same pathological type accounts for 88.9%in UCD without BO patients.The predominantly pathological type(57.1%)was plasma cell variant in the MCD without BO group.Oral ulcers presented in all patients in BO group but were relieved after the mass resection and immunomodulatory therapy,but the pulmonary symptoms were still progressively aggravated.Thoracoscopic mass excision was the main treatment for UCD without BO patients while chemotherapy,immunomodulatory and targeted therapy were commonly used for MCD without BO treatment.Conclusion The age,clinical symptom,laboratory finding,lung function,imaging manifestation,pathology,treatment and prognosis were different among the three groups.This classification could improve clinical understanding of the disease.