女性原发性生殖系统非霍奇金淋巴瘤的临床特征分析

Analysis of clinical features of primary female genital system non-Hodgkin′s lymphoma

目的探讨女性原发性生殖系统非霍奇金淋巴瘤的临床特征。方法回顾性分析解放军总医院2010年1月-2016年1月确诊并住院治疗的19例女性原发性生殖系统非霍奇金淋巴瘤患者的临床病例。结果19例患者中以年龄<60岁人群为主,初诊时多未见贫血发生,月经状态无特殊,超过70%的患者无典型症状且无大型肿物,Ann Arbor分期主要为ⅣE期,检出12例;原发或受侵部位以卵巢为主,检出7例;弥漫大B细胞淋巴瘤是其主要病理类型,检出13例;11例患者国际预后指数(International Prognstic Index,IPI)评分多为中高危。诊断方法中,13例患者为手术确诊,6例通过超声引导下穿刺活检确诊。采用的主要治疗方案包括R-CHOP或CHOP、EPOCH、MINE、ESHAP等,其中7例同时给予阿糖胞苷+甲氨喋呤+地塞米松鞘内注射预防治疗;全部患者均接受3~20个疗程化疗,其中9例完全缓解,7例部分缓解,3例病情进展。5例患者化疗后接受放射治疗,1例接受内照射治疗。随访时间为7~60个月(5年),中位随访时间为20.5个月,19例患者1年、3年和5年总生存率分别为89.47%、70.59%和58.33%。19例患者在治疗期间2例死亡,死亡原因为感染、中枢神经系统进展;5年总死亡12例。结论女性原发性生殖系统非霍奇金淋巴瘤较罕见,发病后无典型症状,确诊时多为晚期,手术、化疗、放疗是其主要治疗手段,5年内生存率相对较低,临床应重视该疾病的早期诊断与治疗,以改善患者预后、延长生存时间、提高生存率。

Objective To investigate the clinical features of primary female genital system non-Hodgkin′s lymphoma(PFGS-NHL)in 19 cases.Methods The clinical data of 19 hospitalized patients with PFGS-NHL from January 2010 to January 2016 were retrospectively analyzed.The medical data questionnaire was designed,and the medical data these of patients were carefully reviewed and recorded.Results All the 19 patients were below 60 years of age.There was no anemia in the initial diagnosis,and there was no special menstrual status.More than 70%of the patients had no typical symptoms and Bulky tumor.The Ann Arbor staging was mainly inⅣE-stage,and a total of 12 cases were detected.The primary or invaded site was mainly ovary,and a total of 7 cases were detected.Diffuse large B-cell lymphoma was the main pathological type,and a total of 13 cases were detected.The International prognstic index(IPI)scores of 11 cases showed that the risk conditions of patients were mostly moderately high risk.Moreover,13 patients were confirmed by surgery,and 6 patients were confirmed by ultrasound-guided puncture biopsy.The main treatment regimens included R-CHOP or CHOP,EPOCH,MINE,ESHAP,etc.Besides,7 cases of them were treated with intrathecal injection of cytarabine,methotrexate and dexamethasone.All patients received 3~20 courses of chemotherapy,including 9 cases of complete remission,7 cases of partial remission and 3 cases of disease progression.After chemotherapy,5 cases received radiation therapy,and 1 case received internal irradiation.The follow-up time was 7~60 months(5 years),and the median follow-up time was 20.5 months.The one-year,three-year and five-year overall survival rates were 89.47%,70.59%and 58.33%,respectively.In all 19 patients,treatment-related death occurred in 2 cases,and the causes of death were infection and central nervous system progression.A total of 12 patients died in 5 years.Conclusion PFGS-NHL was rare,and there was no typical symptom after onset,and most of the patients were in late stage at the time of diagnosis.Surgery,chemotherapy,radiotherapy were the main treatment methods,and the survival rate within 5 years was relatively low.Early diagnosis and treatment of the disease may improve the prognosisand increase the survival rate.