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木村病:20例中国患者的临床特征、治疗及预后

Kimura's disease: clinical characteristics, management and outcome of 20 cases from China.

作者信息

Zhang Yuxin, Bao Huijie, Zhang Xinyu, Yang Fei, Liu Yang, Li Huijuan, Lu Jin, Liu Yanying

机构信息

Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing, China.

Department of Rheumatology and Immunology, The second Hospital of Chifeng, China.

出版信息

Clin Exp Rheumatol. 2022 Mar;40(3):532-538. doi: 10.55563/clinexprheumatol/lahgfo. Epub 2021 Mar 22.

Abstract

OBJECTIVES

To explore the clinical characteristics, diagnosis and the therapeutic effect of Kimura's disease (KD).

METHODS

Clinical data of 20 patients with pathologically confirmed KD admitted to Peking University People's Hospital from June 2000 to June 2019 were analysed. A total of 20 confirmed KD patients were enrolled in the study, 18 male and 2 female, with age-onset ranging from 2 to 58 years.

RESULTS

The masses appear as focal, painless, and immovable with an unclear boundary. The most common predilection is head-neck region (n=15, 75%). 15 patients showed peripheral blood eosinophilia. 14 of 14 patients presented with increased serum IgE level. The prominent pathological characteristic is marked lymphoid hyperplasia accompanied by various degrees of vascular hyperplasia and eosinophil infiltration. Among the 20 patients, 12 experienced recurrence of disease after treatment (surgical resection alone: 9/9; oral corticosteroids combined with immunosuppressants: 1/3; surgical resection followed by oral corticosteroids combined with immunosuppressants: 2/6).

CONCLUSIONS

KD should be considered when the patient presents with head-neck swellings and lymphadenopathy, accompanied by an increase of IgE and eosinophil. Compared with surgery alone, combined therapy seems to be a promising treatment option to reduce the recurrence rate.

摘要

目的

探讨木村病(KD)的临床特征、诊断及治疗效果。

方法

分析2000年6月至2019年6月北京大学人民医院收治的20例经病理确诊的KD患者的临床资料。本研究共纳入20例确诊的KD患者,其中男性18例,女性2例,发病年龄2至58岁。

结果

肿块表现为局灶性、无痛、固定且边界不清。最常见的好发部位是头颈部(n = 15,75%)。15例患者外周血嗜酸性粒细胞增多。14例患者血清IgE水平升高。突出的病理特征是明显的淋巴样增生伴不同程度的血管增生和嗜酸性粒细胞浸润。20例患者中,12例治疗后疾病复发(单纯手术切除:9/9;口服糖皮质激素联合免疫抑制剂:1/3;手术切除后口服糖皮质激素联合免疫抑制剂:2/6)。

结论

当患者出现头颈部肿胀和淋巴结病,伴有IgE和嗜酸性粒细胞升高时,应考虑KD。与单纯手术相比,联合治疗似乎是降低复发率的一种有前景的治疗选择。

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