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目的探讨女性生殖系统郎格罕氏细胞增生症的临床表现特点、诊断及治疗方法。方法回顾性分析我院1989年1月至2012年12月收治的女性生殖系统郎格罕氏细胞增生症4例患者的临床资料,总结该病的临床特点。结果①本组患者发病年龄范围23~70岁,平均年龄43.5岁;②临床表现:2例主要表现为外阴瘙痒伴溃疡形成,1例表现为不规则阴道流血,1例表现为外阴赘生物;4例中3例仅累及外阴,1例累及阴道和宫颈;③均依靠术前组织活检明确诊断,2例仅行手术治疗,1例术后补充放疗,1例术前化疗3次;④术后病理诊断及免疫组化:Langerhans组织细胞增生,CD1a、S-100、CD68、Lagerin均阳性。结论女性生殖系统郎格罕氏组织细胞增生症发病率低,无特异临床症状,病变可以累及外阴,宫颈,子宫内膜和卵巢;诊断主要靠活检病理及免疫组化检查;治疗方法主要以手术及放化疗为主。
Objective To investigate the clinical features, diagnosis and treatment of Langerhans cell hyperplasia in female reproductive system. Methods The clinical data of 4 patients with Langerhans cell hyperplasia in female reproductive system admitted to our hospital from January 1989 to December 2012 were analyzed retrospectively. The clinical features of the disease were summarized. Results ① The age of patients in this group ranged from 23 to 70 years old, with an average age of 43.5 years. ② Clinical manifestations: genital pruritus with ulceration occurred in 2 cases, irregular vaginal bleeding in 1 case and genital neoplasm in 1 case. 4 cases, 3 cases involving only the vulva vulva, 1 case involving the vagina and cervix; ③ rely on preoperative biopsy to confirm the diagnosis, 2 cases were only surgical treatment, 1 case of postoperative radiotherapy, 1 case of preoperative chemotherapy 3 times; ④ surgery Pathological diagnosis and immunohistochemistry: Langerhans cell proliferation, CD1a, S-100, CD68, Lagerin were positive. Conclusions The incidence of Langerhans cell histiocytosis in the female reproductive system is low and has no specific clinical symptoms. The lesions can affect the vulva, cervix, endometrium and ovary. The diagnosis mainly depends on the biopsy and immunohistochemistry. The treatment is mainly performed by surgery And radiotherapy and chemotherapy based.