19例以头颈部肿块为主要表现的木村病临床诊治体会
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1.湖南师范大学附属第一医院(湖南省人民医院);2.南华大学附属南华医院

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The Clinical Diagnosis and Treatment Experience of 19 Cases of Kimura's Disease with Head and Neck Masses as The Main Manifestation
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    摘要:

    摘要:目的:探讨分析木村病(Kimura's Disease)的临床特征及诊治经过,为临床诊疗提供参考。方法:回顾性分析了湖南省人民医院、南华大学附属南华医院2006年1月~2024年6月,19例经病理检查诊断为KD患者的病史、实验室检查、影像学检查、病理资料、治疗方案以及预后,并通过Pubmed查阅相关文献了解KD研究的最新进展。结果:1) 临床特征:19例患者临床表现为头颈部肿块,部分患者合并其他部位肿块,包括腋窝淋巴结,锁骨下淋巴结,腹股沟淋巴结,以及上臂肿块。2)辅助检查提示:外周血嗜酸性粒细胞比例或计数增高13例(68.4%,13/19)。19例患者行超声检查,表现为边界清楚的低回声或无回声肿块。18例患者行CT检查,表现为均匀低密度肿块,边界清,呈均匀轻~中度强化。3)病理组织学特点:大量嗜酸性粒细胞浸润、嗜酸性微脓肿形成、淋巴滤泡增生、滤泡间区扩大以及滤泡溶解。4)预后:18例患者行手术治疗,1例患者行糖皮质激素+环磷酰胺治疗。19例患者均获得随访,随访时间为1~10年。6例患者在原切口部位存在肿块复发(31.6%,6/19)。其中5例,予以再次手术治疗,均得到控制,其中1例患者辅以放射治疗,总剂量30Gy,随访3年未复发。1例患者糖皮质激素+环磷酰胺治疗2周后肿块消退,停药5月后复发,予以手术治疗后随访4年未复发。所有患者均未因复发而死亡。结论:木村病是一种外周血嗜酸性粒细胞升高、血清IgE升高的Th2型炎症疾病,表现为无痛性肿块,以头颈部最为常见。治疗方案以手术治疗为主,KD具有术后易复发的特性,术后辅以放疗可减少复发。复发患者可予以糖皮质激素及免疫治疗。针对Th2型炎症细胞因子的生物制剂可能为KD的治疗带来突破。

    Abstract:

    Abstract: Objective: To discuss the clinical features and treatment experience of Ki-mura’s disease and improve the diagnosis and treatment of this disease. Methods: A retrospective analysis was conducted on the clinical data of Hunan Provincial People's Hospital and Nanhua Hospital Affiliated to University of South China. From January 2006 to June 2024, the medical history, laboratory tests, imaging examinations, patho-logical data, treatment plans, and prognosis of 19 patients diagnosed with KD by pathological examination were collected. Relevant literatures were consulted through Pubmed to understand the latest progress in KD’s research. Results:1) Clinical fea-tures: Nineteen patients presented with head and neck mass, and some patients had combined mass in other areas, including axillary lymph node, subclavian lymph node, inguinal lymph node and upper arm mass.. 2) Auxiliary examinations showed an in-crease in the proportion or count of peripheral blood eosinophils in 13cases (68.4%, 13/19). Ultrasound examinations were performed in 19 patients, showing a low-echo or anechoic mass with clear boundaries. CT examinations were performed in 18 pa-tients, showing a uniform low-density mass with clear boundaries and uniform mild to moderate enhancement.3) Histopathological features: massive eosinophil infiltration, eosinophilic micro-abscess formation, lymphoid follicle hyperplasia, enlargement of interfollicular area, and follicular lysis.4) Prognosis:Eighteen patients underwent surgical treatment, and one patient received treatment with corticosteroids and cyclo-phosphamide. All 19 patients were followed up, with a follow-up ranging from 1 to 10 years. Six patients had a recurrence of a mass at the original incision site (31.6%, 6/19). Among them, 5 cases underwent reoperation, all cases were controlled, and one case received adjuvant radiotherapy with a total dose of 30 Gy, and there was no re-currence after a follow-up of 3 years. One patient showed a relief in the mass after 2 weeks of treatment with glucocorticoids and cyclophosphamide. The mass recurred 5 months after stopping the medication. Then the patient underwent a surgical treat-ment, there was no recurrence during a 4-year follow-up. None of the patients died due to recurrence. Conclusion: Kimura's disease is a Th2-type inflammatory disease characterized by increased peripheral blood eosinophils and elevated serum IgE lev-els, presenting as painless masses, most commonly in the head and neck region. The treatment mainly involves surgical intervention, but Kimura's disease has a tendency to recur postoperatively; adjuvant radiotherapy can help reduce recurrence. In patients with recurrence, corticosteroids and immunotherapy may be administered. Biologics targeting Th2-type inflammatory cytokines may offer breakthroughs in the treatment of Kimura's disease.

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  • 收稿日期:2024-11-05
  • 最后修改日期:2025-01-01
  • 录用日期:2025-01-03
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