伴单克隆免疫球蛋白边缘带淋巴瘤三例报告及文献复习

Marginal zone lymphoma with monoclonal immunoglobulin： three eases report and literaturesreview

目的探讨伴单克隆免疫球蛋白（Mclg）边缘带淋巴瘤（MZL）患者的临床特征和治疗方法。方法收集2007年1月至2014年12月3例伴McIg的MZL患者资料，结合文献报道的36例患者资料进行回顾性分析。结果39例患者中男女比例为1．05：1，平均年龄（65．1±12．3）岁。黏膜相关淋巴组织淋巴瘤（MALTL）28例（71．8％），结内MZL9例（23．1％），脾MZL2例（5．1％）。早期患者9例（23．1％），晚期患者30例（76．9％）。首发症状以皮肤紫癜、周围神经病等非占位性表现常见（65．5％，19／29）。13例（33．3％，13／39）伴自身免疫现象，以干燥综合征最多见。MALTL以非胃肠道型为主（60．7％，17／28）。伴有的McIg以IgM型最多见（82．0％，32／39），余依次为IgA、K-轻链、IgG和双克隆型。血浆MclgM水平为（25．55±21．31）g／L，晚期患者明显高于早期患者[（29．85±20．60）g／L对（3．23±2．95）g／L，P=-0．008]。30例患者接受2-8个疗程化疗，完全缓解（CR）率56．0％，总反应率92．0％；中位随访10个月，3年无疾病进展生存率和总生存率分别为44．7％和76．5％。含和不含利妥昔单抗化疗组患者的总反应率为100．0％和78．6％，CR率为63．6％和50．O％，但差异均无统计学意义（P值均〉O．05）。MclgM型患者CR率明显高于非MclgM型者（P=0．026）；治疗后血浆McIgM水平较治疗前明显下降（P=0．002）。结论伴Mclg的MZL好发于60岁以上老年人，诊断时分期较晚，易伴发自身免疫现象，可能是MZL的一种独特亚型。非胃肠道型MALTL更易伴发Mclg，多见MclgM型，其他免疫球蛋白型少见。MZL患者接受含利妥昔单抗的治疗方案可能疗效会更好。

Objective To investigate the clinical features and treatment in patients of marginal zone lymphoma （MZL） with monoclonal immunoglobulin （McIg）. Methods 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. Results 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 MclgM （82.0%）; the others with McIgA, Mcn- 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/L vs （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 MclgM was significantly higher than that with non-MclgM （P=0.026）. The plasma MclgM level decreased after chemotherapy （P=0.002）. Conclusion The MZL with Mclg, 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 Mclg, MclgM was more common and other Mclg rare. Rituximab-included regimen produced a better therapeutic response.