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30例不同类型胸部受累Castleman病的临床分析

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

作者信息

Zhang X X, Jiang L, Wang C Y, Gu Y Y, Li L G, Xia T T, Huang Y, Huang P K, Zhang Q L

机构信息

National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou Institute of Respiratory Health, Guangzhou 510120, China.

Department of Respiratory and Critical Care Medicine, Huhhot First Hospital, Huhhot 010010, China.

出版信息

Zhonghua Xue Ye Xue Za Zhi. 2020 Feb 14;41(2):149-156. doi: 10.3760/cma.j.issn.0253-2727.2020.02.012.

Abstract

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. 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. 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 (27.20±3.76) ys and (37.17±2.87) ys; =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 group[short diameter (1.83±0.51) cm (4.73±1.63) cm and (3.62±0.26) cm; =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. 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.

摘要

为提高对不同类型胸部受累的Castleman病(CD)的临床认识,包括其临床特征、影像学和病理学表现、诊断及当前治疗策略。对2009年6月至2019年5月在广州医科大学附属第一医院住院的30例诊断为胸部受累的CD患者进行回顾性分析。根据临床资料将患者分为三组进行后续分析:合并闭塞性细支气管炎(BO)的CD、不合并BO的单中心Castleman病(UCD)和不合并BO的多中心Castleman病(MCD)。30例患者中,5例(16.7%)诊断为BO,18例(60.0%)为不合并BO的UCD,7例(23.3%)为不合并BO的MCD。不合并BO的MCD患者的平均年龄显著高于合并BO的患者及不合并BO的UCD患者[(49.29±5.39)岁对(27.20±3.76)岁和(37.17±2.87)岁;分别为P = 0.005和0.034]。肺部症状在BO组(100%)和不合并BO的MCD组(71.4%)中常见,而不合并BO的UCD组未见肺部症状。关键的实验室异常表现为红细胞沉降率(ESR)升高(BO组40%,不合并BO的MCD组57.1%)和低氧血症(BO组60%,不合并BO的MCD组28.6%)。不合并BO的MCD组其他异常实验室表现包括贫血和IgG升高(均为57.1%)。值得注意的是,BO组所有患者肺功能检查均有极重度混合性通气功能障碍。CT扫描显示BO组肺实质受累(100%),不合并BO的UCD组以孤立性肺结节为特征(11.1%),不合并BO的MCD组以双肺弥漫性病变为特征(57.1%)。不合并BO的MCD组淋巴结大小明显小于BO组和不合并BO的UCD组[短径(1.83±0.51)cm对(4.73±1.63)cm和(3.62±0.26)cm;分别为P = 0.006和0.011]。BO组所有患者(100%)病理类型为透明血管型,不合并BO的UCD患者中该病理类型占88.9%。不合并BO的MCD组主要病理类型(57.1%)为浆细胞型。BO组所有患者均出现口腔溃疡,但肿块切除及免疫调节治疗后缓解,但肺部症状仍逐渐加重。胸腔镜肿块切除是不合并BO的UCD患者的主要治疗方法,而化疗、免疫调节和靶向治疗常用于不合并BO的MCD的治疗。三组在年龄、临床症状、实验室检查、肺功能、影像学表现、病理、治疗及预后方面存在差异。这种分类有助于提高对该疾病的临床认识。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/647f/7357939/c70ac6e3c307/cjh-41-02-149-g001.jpg

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