非胃肠道的黏膜相关淋巴组织淋巴瘤48例临床分析

Clinical Analysis of Non-gastrointestinal MALT Lymphoma in 48 Patients

目的分析非胃肠道黏膜相关淋巴组织(MALT)淋巴瘤患者的临床特点,以提高临床诊断及治疗水平。方法回顾性分析北京协和医院2000年1月至2011年7月经病理证实明确诊断为非胃肠道MALT淋巴瘤的48例患者的资料。结果 48例患者中男16例,女32例;诊断时的年龄为21～76岁,平均55.4岁。发病部位为肺23例,涎腺9例,甲状腺8例,眼及附属器3例,其他部位5例,同时发生于两个部位的5例。临床表现为无症状者27例,有非特异性全身症状者13例。实验室检查中,8例低中度贫血,6例乳酸脱氢酶水平升高,19例血沉加快。影像学检查提示20例淋巴结肿大。6例患者合并自身免性疫疾病,其中3例为涎腺病例。肺部受累患者确诊经支气管活检获得病理诊断3例,CT引导下经皮肺穿刺11例,外科肺活检9例。肺外受累患者确诊均经外科活检。23例肺部受累患者中,有1例未经处理,其余22例接受各种组合治疗(单纯手术3例,手术联合化疗6例,单纯化疗13例);25例肺外受累患者中,11例患者接受单纯手术治疗,10例患者接受手术+化疗联合治疗,3例接受单独的化疗,此外还有1例患者予以手术+化疗+放疗联合治疗。随访4～133个月,平均46.7个月,肺部受累患者1例死于肺部感染,1例死因不详,其余患者均病情平稳。结论非胃肠道MALT淋巴瘤常见于中老年女性,肺、涎腺和甲状腺为其好发部位,临床上多无明显症状或仅限于非特异性全身症状,部分合并自身免疫性疾病。CT引导下经皮穿刺肺活检和手术活检获取病理组织有助于明确诊断。本淋巴瘤的预后较好,部分患者可经外科手术治愈。

Objective To investigate the clinical features of non-gastrointestinal mucosa-associated lymphoid tissue （MALT） lymphoma. Methods Forty-eight pathologically proved cases of non- gastrointestinal MALT lymphoma, admitted into Peking Union Medical College Hospital from January 2000 to July 2011 ,were retrospectively analyzed. Results There were 32 females and 16 males. The median age at diagnosis was 55.4 years old （range,21-76 years）. The most commonly affected sites were lung,salivary glands, thyroid and ocular adnexa. In 5 cases, the lymphoma presented at multiple mucosal sites. 27 patients were asymptomatic while 13 had non-specific symptoms. Blood test showed mild or moderate anemia in 8 cases, elevated erythrocyte sedimentation rate in 19 cases, and elevated lactate dehydrogenase in 6 patients. Imaging examinations revealed enlarged lymph nodes in 20 patients. 6 patients had a history of Sjoren＇ s syndrome, in whom 3 cases were salivary gland diseases. In the patients with lung involvement, pathological diagnosis was obtained by bronchial biopsies in 3 cases, by CT-guided percutaneous lung biopsies in 11 cases,and by surgical biopsies in 9 cases. While in the patients without lung involvement, pathological diagnosis was obtained all by surgical biopsies. Of the 23 patients with lung involvement, 1 remain untreated, while 22 received various combinations of treatment （ surgery alone in 3 patients, surgery plus chemotherapyin 6 patients, and chemotherapy alone in 13 patients）. Of the 25 patients without lung involvement, 11 patients received surgery alone, 10 patients received surgery plus chemotherapy, 3 patients received chemotherapy alone, and 1 patient received surgery plus chemotherapy and radiotherapy. 46 patients remained alive during the median follow-up of 46.7 months （range,4-133 months）. While 1 patient with lung involvement died from unknown causes, another 1 patient with lung involvement died from lung infection. Conclusions Non-gastrointestinal MALT lymphoma tends to occur in old-aged females, and commonly occurs in lung, salivary gland and thyroid sites. Most patients are asymptomatic or have only non- specific symptoms. CT-guided percutaneous lung biopsies and surgical biopsies are helpful to the diagnosis. Prognosis for this lymphoma tends to be indolent.