Wang Fang, Han Xue, Bai Beibei, Wang Chunjian, Chen Ye
Department of Hematology, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China.
Zhonghua Xue Ye Xue Za Zhi. 2016 Jan;37(1):39-44. doi: 10.3760/cma.j.issn.0253-2727.2016.01.008.
To investigate the clinical features and treatment in patients of marginal zone lymphoma (MZL)with monoclonal immunoglobulin (McIg).
The clinical data of MZL patients with McIg, including 3 cases diagnosed and treated in Beijing Anzhen Hospital from Jan 2007 to Dec 2014 were retrospectively studied, meanwhile 36 patients searched from literatures were reviewed.
Of a total of 39 patients, the ratio of male and female was 1.05∶1 with an average age of 65.1± 12.3 years old. 28 cases (71.8%)were with mucosa associated lymphoid tissue lymphomas (MALTL), 9 cases (23.1% )with nodal marginal zone lymphoma, and 2 cases (5.1%)with splenic marginal zone lymphoma. Nine cases (23.1% )were in the early stage, 30 cases (76.9%)in the advanced stage. The common initial symptom was non-mass lesions (65.5%), such as skin purpura, peripheral neuropathy; 13 patients (33.3% )were accompanied by autoimmune phenomenon, and most were with Sjogren's syndrome. Among MALTL patients, the common primary lesion was in non- gastrointestinal tract (17 cases, 60.7%). Most of patients with McIg were one with McIgM (82.0%); the others with McIgA, Mcκ-light chain, McIgG and double McIg. The level of plasma McIgM was (25.55±21.31)g/L, which was higher in advanced stage patients than in early stage ones [(29.85±20.60)g/Lvs (3.23±2.95)g/L,P= 0.008]. The complete remission (CR)rate was 56.0% and the overall response rate (ORR)92.0%, respectively in 30 patients treated by chemotherapy. At a median follow- up of 10 months, the 3- year progression free survival and the 3-year overall survival were 44.7% and 76.5%, respectively. The rates of ORR and CR in the patients received rituximab- included regimen were seemly better than those without rituximab one (100.0%vs 78.6%, 63.6%vs 50.0%;P>0.05), but no statistic differences were found. The CR rate in patients with McIgM was significantly higher than that with non- McIgM (P=0.026). The plasma McIgM level decreased after chemotherapy (P=0.002).
The MZL with McIg, perhaps a kind of unique subtype, usually occurred in 60 years or older patients. It was often diagnosed in patients of advanced stage and susceptible to autoimmune phenomenon. MALTL in non- gastrointestinal tract was more prone to find McIg. In MZL patients with McIg, McIgM was more common and other McIg rare. Rituximab-included regimen produced a better therapeutic response.
探讨伴有单克隆免疫球蛋白（McIg）的边缘区淋巴瘤（MZL）患者的临床特征及治疗情况。
回顾性分析2007年1月至2014年12月在北京安贞医院确诊并治疗的3例伴有McIg的MZL患者的临床资料，同时复习文献检索出的36例患者资料。
39例患者中，男女比例为1.05∶1，平均年龄65.1±12.3岁。28例（71.8%）为黏膜相关淋巴组织淋巴瘤（MALTL），9例（23.1%）为结内边缘区淋巴瘤，2例（5.1%）为脾边缘区淋巴瘤。9例（23.1%）处于早期，30例（76.9%）处于晚期。常见的首发症状为非肿块性病变（65.5%），如皮肤紫癜、周围神经病变；13例患者（33.3%）伴有自身免疫现象，多数为干燥综合征。在MALTL患者中，常见的原发部位为非胃肠道（17例，60.7%）。多数伴有McIg的患者为伴有McIgM（82.0%）；其他为伴有McIgA、Mcκ轻链、McIgG及双McIg。血浆McIgM水平为（25.55±21.31）g/L，晚期患者高于早期患者[（29.85±20.60）g/L对（3.23±2.95）g/L，P = 0.008]。30例接受化疗的患者完全缓解（CR）率为56.0%，总缓解率（ORR）为92.0%。中位随访10个月时，3年无进展生存率和3年总生存率分别为44.7%和76.5%。接受含利妥昔单抗方案治疗的患者ORR和CR率似乎优于未接受利妥昔单抗方案者（100.0%对78.6%，63.6%对50.0%；P>0.05），但差异无统计学意义。伴有McIgM的患者CR率显著高于不伴有McIgM者（P = 0.026）。化疗后血浆McIgM水平下降（P = 0.002）。
伴有McIg的MZL可能是一种独特的亚型，通常发生于60岁及以上患者。常于晚期患者中诊断出，易出现自身免疫现象。非胃肠道的MALTL更容易发现McIg。在伴有McIg的MZL患者中，McIgM更常见，其他McIg少见。含利妥昔单抗方案产生更好的治疗反应。
