13例嗜酸性粒细胞增生性淋巴肉芽肿的临床特征、病理学表现及文献复习

Clinical characteristics and pathological features of Kimura disease: report of 13 case and review of literature

目的 :探讨嗜酸性粒细胞增生性淋巴肉芽肿(Kimura病)的临床特征、病理学表现,并总结其诊断及治疗经验,以提高医师对该病的认识及诊断水平。方法:收集13例Kimura病患者的临床资料、病理组织学形态及治疗情况进行回顾性分析,结合国内外已报道的Kimura病文献,总结该病的临床病理学特征及诊疗方法。结果:13例Kimura病患者均为男性,平均年龄为(41±4)岁,平均病程为(35±11)个月。患者的临床表现为,在不同部位呈进行性增大、无痛性肿块,其中3例伴有躯干和(或)四肢散在丘疹伴瘙痒;4例肿块单发,9例肿块多发,其中最大直径为10.0 cm;9例获得血常规检查资料,结果均显示外周血嗜酸性粒细胞增多;4例接受免疫球蛋白检查,均提示血清Ig E升高;2例接受骨髓穿刺,提示骨髓增生活跃,嗜酸性粒细胞明显浸润。组织形态学上以增生的淋巴组织为主,生发中心扩大、套区结构明显,可见程度不一的淋巴组织增生、淋巴滤泡形成或增生及纤维组织增生,生发中心扩大、套区结构明显,淋巴滤泡间可见小血管增生;间质有大量成熟嗜酸性粒细胞浸润聚集,部分形成嗜酸性微小脓肿。7例患者单纯接受肿块切除手术治疗,余6例患者还接受激素或放射等治疗;7例患者获得随访4~96个月,其中有5例复发。结论:Kimura病是一种良性病变,其发病率低且临床表现不具有特异性,容易误诊及漏诊;掌握其临床特点和组织学形态对提高临床医师的诊断及鉴别诊断能力具有十分重要的意义。

Objective： To investigate the clinical and pathological features of Kimura’s disease（KD） for improving its diagnosis and treatment experiences. Methods： The clinical data and histological morphology of 13 cases of Kimura disease were retrospectively analyzed, with review of literature.Clinicopathological features, diagnosis and treatment of Kimura disease were summarized. Results： All the patients were male, with mean age of（41±4） years and average duration of（35±11） months. The clinical manifestations of KD were progressive and painless tumors in different sites, including 3 cases with trunk and/or limbs scattered with papules with itching, 4 cases with single tumor nodule, 9 cases with multiple tumors. The largest tumor had a diameter reaching 10.0 cm. Blood examination showed increased peripheral blood eosinophils in all the cases. Four cases had immunoglobulin Ig E test performed, and it showed increased level of serum Ig E. And 2 cases had bone marrow puncture performed, which revealed active proliferation of bone marrow and evident infiltration of eosinophils. Histopathologically, they mainly composed of hyperplastic lymphoid tissue, expansion of germinal center, obvious mantle zone, lymphoid follicle formation and fibrous tissue hyperplasia;small vascular hyperplasia can be seen between lymph follicles. Marked infiltration and accumulation of mature eosinophils were seen in interstitial tissue,and eosinophilic tiny abscesses were formed.Seven cases received surgical resection only, and the other 6 cases received treatment such as hormones or radiation in addition. Seven patients were followed up for 4-96 months and 5 had relapse.Conclusions： Kimura disease is a kind of benign lymphoproliferative lesion, and it is important to master its clinical features and histological morphology for improving its diagnosis.