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Title: [Diagnosis and treatment of 41 cases of head and neck Castleman's disease].
Author: Xue JF, Hao Q, Zhang YM, Cao PD, Song SP, Hu RF, Jin S.
Journal: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi; 2018 Aug 07; 53(8):610-614. PubMed ID: 30122000.
Abstract:
Objective: To explore the diagnosis and treatment of head and neck Castleman's disease (CD), and to improve the understanding of the disease. Methods: The clinical data of 41 patients with head and neck CD treated from January 2007 to July 2017 in the First Affiliated Hospital of Zhengzhou University were analyzed retrospectively. The patients were divided into two groups: localized CD (LCD, n=27) and multicentric CD (MCD, n=14). The clinical manifestations, laboratory examinations, operations, pathological findings and follow-up data were analyzed. Results: LCD was characterized by asymptomatic enlargement of the single lymph node with mild clinical symptoms. The main pathological type was hyaline vascular type, which can be cured by surgical treatment. MCD accompanied by fever 9 cases(64.3%), anemia 8 cases (57.1%), splenomegaly 7 cases (50.0%), respiratory symptoms 6 cases (42.9%), with multiple enlarged superficial and deep lymph nodes with pain, pleural effusion, and edema of lower extremity, C-reaction protein increased in 8 cases (57.1%), hypoproteinemia 8 cases(57.1%), globulin increased 7 cases (50.0%) and neutropenia 6 cases(42.9%). The main pathological type was plasma cell type in 7 cases (50.0%), and also there were 4 cases of hyaline vascular type and 2 cases of mixed type. Chemotherapy with or without rituximab was the main treatment. Most patients received complete or partial remission except for 2 patients who died of lung infection due to chemotherapy. Conclusions: The diagnosis of head and neck CD depends on pathological examination. LCD shows mild symptoms and good prognosis after surgery. However, clinical manifestations of MCD are complex, with relatively poor prognosis after comprehensive treatment. 目的： 探讨头颈部Castleman病的临床特点和治疗方法，提高头颈部Castleman病的诊治水平。 方法： 回顾性分析2007年1月至2017年7月经郑州大学第一附属医院病理确诊的41例头颈部Castleman病患者的临床资料。根据临床分型分为两组，局灶型组(n＝27)和多中心型组(n＝14)，对其临床表现、实验室检查、手术方式、病理学检查和随访资料进行统计学分析。 结果： 局灶型常表现为无症状的单个淋巴结肿大，临床症状轻微，病理以透明血管型为主，通过手术治疗可治愈。多中心型表现为全身多发浅表及深部淋巴结肿大，本组患者表现为发热9例次(64.3%)、贫血8例次(57.1%)、脾肿大7例次(50.0%)、咳嗽咳痰或呼吸困难6例次(42.9%)，伴淋巴结疼痛、胸腹腔积液、颜面部及下肢水肿、C反应蛋白升高8例次(57.1%)、低蛋白血症8例次(57.1%)、球蛋白增多7例次(50%)、白细胞增多6例次(42.9%)等，病理以浆细胞型为主7例次(50.0%)，4例为透明血管型，2例为混合型，7例为浆细胞型。主要采用联合或不联合利妥昔单抗化疗方案，2例因化疗引起肺部感染死亡，其余患者获得完全或部分缓解。 结论： 头颈部Castleman病需病理检查确诊，局灶型患者临床症状轻微，手术治疗效果良好且预后好，多中心型患者临床症状复杂，需综合治疗，预后相对较差。.

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