血管淋巴样增生伴嗜酸性粒细胞浸润合并肾损害三例并文献复习

目的分析血管淋巴样增生伴嗜酸性粒细胞浸润（ALHE）合并肾损害的临床病理特点。方法分析北京大学第一医院肾内科诊断的3例ALHE合并肾损害的临床资料,并对其淋巴结及肾活检组织进行病理学观察。结果3例患者均为男性,以头颈部皮下肿块伴大量蛋白尿和肾功能损害为主要临床表现,外周血嗜酸性粒细胞增多和IgE增高,2例有血免疫球蛋白及补体异常。糖皮质激素及环磷酰胺治疗有效,其中例3患者停药后病情反复。病理学改变：淋巴结和肾组织内可见突出的小血管增生,内皮细胞增生肥大呈上皮细胞样,管腔狭窄或闭塞,伴有淋巴细胞、浆细胞及较多的嗜酸性粒细胞浸润。免疫组化染色可见CD31、CD34及F8在增生的血管内皮细胞呈阳性。肾脏病变主要累及肾小管间质,其中例1患者的肾脏病理改变为Ⅰ期膜性肾病。结论ALHE合并肾损害在我国并不罕见,其临床病理特征支持其为一种与变态反应有关的血管及淋巴组织增生性疾病。

Angiolymphoid hyperplasia with eosinophilia involving the kidney: a report of three cases with literature review

OBJECTIVE: To report the clinical and pathological characteristics of renal involvement in angiolymphoid hyperplasia with eosinophilia (ALHE). METHODS: Three cases of ALHE with renal involvement were diagnosed in our hospital. Routine pathological examination and immunohistochemical study of CD(31), CD(34) and F(8) of lymph node and renal biopsy specimens were performed and the clinical and pathological features were analysed. RESULTS: All three patients were male. Subcutaneous mass in the neck and head region accompanied with proteinuria and renal function impairment were the clinical characteristics of the three patients. Peripheral blood eosinophilia with elevated IgE level was detected in all the 3 patients. The level of serum immunoglobulins and complements were abnormal in 2 of them. They were sensitive to corticosteroid and cyclophosphamide therapy, but one case showed a recurrence after the withdrawal of immunosuppressive agents. Histopathological examination of lymph node and renal tissue showed a prominent proliferation of small vessels lined with hypertrophic epitheloid endothelial cells, which led to a narrowed or even closed vascular lumen. Diffuse infiltration of eosinophils, lymphocytes and plasma cells was identified around the hyperplastic vessels. With immunohistochemical study, CD(31), CD(34) and F(8) were positive in hyperplastic endothelial cells. Renal lesions were mainly distributed in tubulointerstitial area, while one case exhibited membranous nephropathy (stage I). CONCLUSION: ALHE with renal involvement is not rare in our country. The clinical and pathological characteristics of ALHE suggested that it might belong to an entity of angiolymphoid hyperplasia associated with allergic response.