探讨木村病的临床诊断学特征。

回顾性分析1例木村病患者的临床特征、实验室检验、基因检测、影像学特征、骨髓穿刺特征和病理学特征。

木村病患者白细胞计数11.65×10⁹/L，嗜酸性粒细胞绝对值3.12×10⁹/L，嗜酸性粒细胞占35.6%；红细胞沉降率32 mm/h；C反应蛋白20 mg/L；颈部淋巴结超声示左颈部可探及数个淋巴结，呈串珠样，皮质增厚，紧邻血管；胸腹部CT及纤维支气管镜检查未见明显异常。骨髓穿刺示骨髓增生活跃，以粒系和红系增生为主，巨核系无明显异常，嗜酸性细胞偏高。颈部淋巴结切除术中见颈前及外侧区多枚肿大淋巴结呈串珠样分布，部分融合成团，质韧，边界欠清，与周围组织粘连。组织病理学检查示淋巴组织大量增生，以淋巴滤泡增生为主，滤泡间小血管管壁增厚伴玻璃样变性，弥漫嗜酸性粒细胞浸润。组织免疫组化染色示：滤泡间区、滤泡散在的CD3和CD20(＋)、生发中心80%Ki-67(＋)、滤泡树突网CD21(＋)、生发中心Bcl-2(－)。

木村病是一种大量淋巴组织增生伴外周血嗜酸性粒细胞升高和血清IgE增高的慢性免疫性疾病。诊断需综合体格检查、实验室检验和影像学检查结果，明确诊断需组织病理学检查。

To investigate the clinical features of Kimura′s disease.

The clinical characteristics, laboratory tests, genetic testing, the features of imaging, bone marrow biopsy and pathology of Kimura′s disease were retrospectively analyzed.

Laboratory tests showed that white blood cell count was 11.65×10⁹/L, eosinophil count was 3.12×10⁹/L, the percentage of eosinophil was 35.6%.Erythrocyte sedimentation rate was 32mm/h, C-reactive protein was 20 mg/L.The ultrasound showed that there were several beaded lymph node which were adjacent to blood vessels on the left neck, and the cortical thickened.Thoraco-abdominal CT and bronchoscopy revealed no obvious abnormalities.Bone marrow biopsy showed myeloid and erythroid hyperplasia.A number of beaded lymph node integration into group, with the surrounding tissue adhesion.Histopathological examination showed that lymphoid hyperplasia with follicular hyperplasia and diffuse eosinophilic infiltration on interfollicular.Immunohistochemical staining showed CD3, CD20, CD21 and 80% of Ki-67 of follicular area were positive, Bcl-2 was negative.

Kimura′s disease is a chronic inflammatory disease characterized by lymphadenia, with high peripheral blood eosinophil count and serum immunoglobulin E (IgE). Its diagnosis relys on physical examination, laboratory tests and imaging findings.Histopathological examination can confirm the diagnosis.