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1例Castleman病罕见病例表现为双肺弥漫性磨玻璃结节:病例报告

A rare case of Castleman disease presented with diffuse ground glass nodules in both lungs: Case report.

作者信息

Guo Xiaotong, Zhu Caixia, Zhang Fen, Chen Juan, Zhang Kedong

机构信息

Department of Respiratory and Critical Care Medicine, The General Hospital of Ningxia Medical University, Yinchuan, China.

Department of Rheumatology, The General Hospital of Ningxia Medical University, Yinchuan, China.

出版信息

Medicine (Baltimore). 2025 Apr 4;104(14):e40681. doi: 10.1097/MD.0000000000040681.

DOI:10.1097/MD.0000000000040681
PMID:40193641
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11977701/
Abstract

RATIONALE

Castleman disease (CD) is a rare chronic lymphoproliferative disorder with unclear etiology and pathogenesis. It is divided into unicentric CD, which involved a single enlarged lymph node or region of lymph nodes, and multicentric CD, which involved multiple lymph node stations. Chest computed tomography (CT) scan is of great value in the diagnosis and differential diagnosis of the disease. CT scan mainly present large soft tissue mass in lungs and multiple mediastinal lymph node enlargement. Multiple ground glass nodules in both lungs are rare in CD patient.

PATIENT CONCERNS

A 48-year-old woman presented with chest tightness, shortness of breath, cough, and sputum. The chest CT scan showed multiple ground glass nodules in both lungs.

DIAGNOSES

Multicentric Castleman disease was diagnosed through biopsies of the mediastinal 4R group, 7th group lymph nodes and the right inguinal lymph nodes.

INTERVENTIONS

Initial treatment with prednisone was administered, but due to the absence of significant radiological improvement on chest CT after 1 month, a systemic chemotherapy was initiated.

OUTCOMES

After 6 cycles of systemic chemotherapy with cyclophosphamide, vincristine, and prednisone, the patient chest CT and clinical symptoms improved. Currently, the patient is still receiving low-dose prednisone and cyclophosphamide orally for long-term maintenance treatment.

LESSONS

CD that presents multiple ground glass nodules in both lungs is rare. It is easily confused with other diseases, identified diagnosis is depend on pathological examination. The accuracy of clinical subtype and histopathogenic type are important for treatment and outcome.

摘要

原理

卡斯特曼病(CD)是一种病因和发病机制不明的罕见慢性淋巴细胞增殖性疾病。它分为单中心型CD,累及单个肿大的淋巴结或淋巴结区域,以及多中心型CD,累及多个淋巴结部位。胸部计算机断层扫描(CT)对该病的诊断和鉴别诊断具有重要价值。CT扫描主要表现为肺部的大软组织肿块和多个纵隔淋巴结肿大。双肺多发磨玻璃结节在CD患者中较为罕见。

患者情况

一名48岁女性出现胸闷、气短、咳嗽和咳痰症状。胸部CT扫描显示双肺多发磨玻璃结节。

诊断

通过对纵隔4R组、第7组淋巴结及右侧腹股沟淋巴结进行活检,诊断为多中心型卡斯特曼病。

干预措施

最初给予泼尼松治疗,但1个月后胸部CT未见明显影像学改善,遂开始全身化疗。

结果

采用环磷酰胺、长春新碱和泼尼松进行6个周期的全身化疗后,患者的胸部CT和临床症状有所改善。目前,患者仍在口服低剂量泼尼松和环磷酰胺进行长期维持治疗。

经验教训

双肺出现多发磨玻璃结节的CD较为罕见。它容易与其他疾病混淆,明确诊断依赖于病理检查。临床亚型和组织病理学类型的准确性对治疗和预后很重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0587/11977701/58f80032ae47/medi-104-e40681-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0587/11977701/912873ee1e8c/medi-104-e40681-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0587/11977701/8cc3f1093399/medi-104-e40681-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0587/11977701/58f80032ae47/medi-104-e40681-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0587/11977701/912873ee1e8c/medi-104-e40681-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0587/11977701/8cc3f1093399/medi-104-e40681-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0587/11977701/58f80032ae47/medi-104-e40681-g003.jpg

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本文引用的文献

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Idiopathic multicentric Castleman disease and associated autoimmune and autoinflammatory conditions: practical guidance for diagnosis.特发性多中心 Castleman 病及相关自身免疫和自身炎症性疾病:诊断的实用指南。
Rheumatology (Oxford). 2023 Apr 3;62(4):1426-1435. doi: 10.1093/rheumatology/keac481.
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IgG4-Related Disease: Mimickers and Diagnostic Pitfalls.IgG4 相关疾病:类似疾病与诊断陷阱。
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Immunoglobulin G4-related disease and idiopathic multicentric Castleman's disease: confusable immune-mediated disorders.
免疫球蛋白G4相关性疾病与特发性多中心Castleman病:易混淆的免疫介导性疾病。
Rheumatology (Oxford). 2022 Feb 2;61(2):490-501. doi: 10.1093/rheumatology/keab634.
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Immunoglobulin G4-Related Disease: What an Allergist Should Know.免疫球蛋白 G4 相关疾病:过敏科医生应知要点。
J Investig Allergol Clin Immunol. 2021 Jun 22;31(3):212-227. doi: 10.18176/jiaci.0633. Epub 2020 Jul 30.
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