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12例胸内castleman病患者的临床分析

Clinical analysis for 12 patients with thoracic Castleman's disease.

作者信息

Shen Chong, Ouyang Ruoyun, Liu Guiqian, Liu Ting

机构信息

Department of Respiratory and Critical Care Medicine, Second Xiangya Hospital, Central South University, Changsha 410011, China.

出版信息

Zhong Nan Da Xue Xue Bao Yi Xue Ban. 2020 Nov 28;45(11):1336-1341. doi: 10.11817/j.issn.1672-7347.2020.190203.

Abstract

OBJECTIVES

To explore the clinical characteristics and differential diagnosis for patients with thoracic Castleman's disease, thus to improve doctor's cognition and therapeutic level for the disease.

METHODS

The clinical data of 12 patients with thoracic Castleman's disease were analyzed retrospectively, including clinical features, laboratory examination, imaging finding, pathological examination, treatment outcome, and prognosis.

RESULTS

All 12 patients were diagnosed by mass or lymph code biopsy. Seven patients with unicentric Castleman's disease were asymptomatic or oligosymptomatic. Of them, the lung imaging features were mostly solitary and well-defined pulmonary hilar soft tissue mass and infrequently invasive mass. Pathologically, 7 patients were all hyaline vascular type. Six patients underwent surgery of complete mass resection, and 1 received chemotherapy because the nidus could not be resected completely. The prognosis of 7 patients was good. The clinical manifestations of 5 patients with multicentric Castleman's disease were diverse with negative prognosis. Their radiological features mainly showed multiple mediastinal/hilum lymph node enlargement, multiple serous cavity effusion, and hepatosplenomegaly, along with multifocal lymphadenopathy. Pathological features of 3 patients' mass were hyaline vascular type, 1 was plasma cell type, and 1 was mixed type. Three patients received comprehensive treatment on the basis of chemotherapy and another 2 patients did not receive any therapy.

CONCLUSIONS

Unicentric Castleman's disease involving the chest is mainly manifested as mediastinal or hilar soft tissue mass with single clinical manifestation, while multicentric Castleman's disease involving the chest is mainly manifested as diffuse mediastinal/hilar lymph node enlargement, multifocal lymphadenopathy and multiple clinical manifestation. For patients with mediastinal/hilum mass or with multifocal lymphadenopathy, Castleman's disease should be considered the differential diagnosis. And early mass or lymph code biopsy is of great significance for diagnosis and treatment of Castleman's disease.

摘要

目的

探讨胸内Castleman病患者的临床特征及鉴别诊断,以提高医生对该疾病的认识及治疗水平。

方法

回顾性分析12例胸内Castleman病患者的临床资料,包括临床特征、实验室检查、影像学表现、病理检查、治疗结果及预后。

结果

12例患者均经肿块或淋巴结活检确诊。7例单中心Castleman病患者无症状或症状轻微。其中,肺部影像学特征多为孤立、边界清晰的肺门软组织肿块,较少见浸润性肿块。病理上,7例均为透明血管型。6例患者行肿块完整切除术,1例因病灶无法完全切除而接受化疗。7例患者预后良好。5例多中心Castleman病患者临床表现多样,预后不良。其影像学特征主要表现为多发纵隔/肺门淋巴结肿大、多浆膜腔积液及肝脾肿大,伴有多部位淋巴结病变。3例患者肿块病理特征为透明血管型,1例为浆细胞型,1例为混合型。3例患者在化疗基础上接受综合治疗,另外2例患者未接受任何治疗。

结论

累及胸部的单中心Castleman病主要表现为纵隔或肺门软组织肿块,临床表现单一,而累及胸部的多中心Castleman病主要表现为弥漫性纵隔/肺门淋巴结肿大、多部位淋巴结病变及临床表现多样。对于纵隔/肺门肿块或多部位淋巴结病变患者,应考虑Castleman病进行鉴别诊断。早期肿块或淋巴结活检对Castleman病的诊断和治疗具有重要意义。

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