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目的 :分析 6例Kimura病肾损害的临床病理特点。 方法 :1998年至 2 0 0 4年经皮淋巴结活检诊断为Kimura病且伴有肾损害 6例 ,分析患者的临床特点、实验室检查、肾活检病理改变及治疗反应。 结果 :① 4例以颈部肿块首发 ,2例以肾病综合征首发 ,6例均有皮下肿块及淋巴结肿大 ,浅表淋巴结累及一个或多个部位 ,2例伴有深部淋巴结肿大。② 5例血嗜酸细胞升高 (占白细胞比例 10 1%～ 4 4 3% ) ,5例血清IgE升高 (10 7 3～988 7IU/ml)。③ 6例肾脏损害均表现为典型肾病综合征 ,无血尿及高血压 ,肾活检病理 5例表现为肾小球系膜增生性病变 ;余 1例为膜性病变 ,其免疫荧光为少量免疫复合物沉积。 5例间质明显嗜酸细胞浸润。④入院前激素治疗均敏感但反复发病 ,入院后均采用激素诱导治疗 ,3例同时行局部淋巴结切除术 ,均获完全缓解。随访 6～ 36个月期间 ,肾功能正常 ,2例肾病综合征反复发作 (未切除淋巴结 ) ,3例同时切除淋巴结者随访期内未复发。 2例皮下肿块复发。 结论 :①青少年男性肾病患者伴有皮下肿块 ,血嗜酸细胞及IgE升高 ,应排除Kimura病。②肾活检病理肾间质嗜酸细胞浸润是Kimura病肾损害的主要特征。③单纯激素治疗敏感但复发率高。
Objective: To analyze the clinicopathological features of 6 cases of Kimura disease. Methods: Percutaneous lymph node biopsy was diagnosed as Kimura disease from 1998 to 2004 with 6 cases of renal damage. The clinical features, laboratory tests, pathological changes of renal biopsy and response to treatment were analyzed. Results: ① 4 cases started with neck mass, 2 cases started with nephrotic syndrome, 6 cases had subcutaneous mass and lymph node enlargement, superficial lymph nodes involved one or more sites, 2 cases with deep lymph nodes. ② 5 cases of blood eosinophilia (accounting for 10 1% ~ 443% of leukocytes), 5 cases of serum IgE increased (107 ~ 988 7IU / ml). ③ The renal lesions in 6 cases all showed typical nephrotic syndrome without hematuria and hypertension. The pathology of renal biopsy manifested as mesangial proliferative lesions in 5 cases. The other 1 cases were membranous lesions with immunofluorescence for a small amount of immune complex Material deposition. 5 cases of interstitial eosinophilic infiltration. ④ Hormone therapy before admission were sensitive but recurrent disease, after admission were treated with hormone therapy, 3 cases underwent simultaneous local lymph node dissection, were completely relieved. During 6 to 36 months of follow-up, renal function was normal, recurrent nephrotic syndrome in 2 (non-excised lymph nodes), and no recurrence in 3 cases with lymph node dissection at the same time. 2 cases of subcutaneous tumor recurrence. Conclusions: ①The juvenile nephropathy patients with subcutaneous tumor, blood eosinophils and IgE increased, Kimura disease should be excluded. ② renal biopsy pathological renal interstitial eosinophilic infiltration Kimura disease is the main feature of renal damage. ③ hormone therapy alone sensitive but high recurrence rate.