16例套细胞淋巴瘤临床病例分析

Clinical analysis of 16 cases of mantle cell lymphoma

目的：探讨套细胞淋巴瘤（MCL）患者的临床特点、治疗与疗效的关系。方法：回顾分析我院16例MCL患者的临床资料,采用MCL国际预后指数（MIPI）和MIPIb（Ki-67）评估,观察短期缓解率和长期疗效,包括2年总生存率及无进展生存率（PFS）,分析临床因素对预后的影响。结果：16例患者中,男10例,女6例,男∶女约为3∶2,临床分期Ⅲ~Ⅳ期约占93.8%,所有患者均接受1个疗程以上的CHOP（E）±美罗华（R）等方案化疗。预后因素分析发现：①虽然MIPI〈5.7、Ki-67≤40%、β2-GM〈3mg/L各组患者分别与MIPI≥5.7、Ki-67〉40%、β2-GM≥3mg/L各组患者的短期缓解率无明显差异,但前3个组患者2年PFS率分别明显高于后3个组（66.7%∶25.0%,63.6%∶19.1%,78.9%∶33.5%,均P〈0.05）;②9例检测SOX-11,阳性率66.7%（6/9）,未发现预后相关性;③CHOP（E）＋R与PAD方案组的短期完全缓解率及2年PFS率均明显优于CHOP（E）方案组（57.1%∶100%∶0,71.4%∶100%∶11.1%,均P〈0.05）;④难治/复发患者中,1例行自体造血干细胞移植及2例行含硼替佐米的PAD方案化疗,均获得长期PFS。结论：MCL患者男性多发,多为Ⅲ~Ⅳ期,MIPI指数、Ki-67、β2-GM对临床预后判断有重要作用;SOX-11预后相关性有待深入研究;CHOP（E）＋R较CHOP（E）疗效好,PAD方案联合美罗华维持治疗对难治/复发患者的疗效显著,有待进一步临床观察。

Objective：To investigate the relationship between the clinical characteristics,therapy and curative efficacy of mantle cell lymphoma（MCL）patients in our hospital.Method：Clinical data of 16MCL patients in our hospital has been retrospectively analyzed.We used MCL international prognostic index（MIPI）,MIPI-biological（Ki-67）and observed the short-term remission rate and long-term curative effect including 2years overall survival and progression-free survival（PFS）to analyze the clinical prognostic factors.Result：Among the 16patients,the ratio of male to female was 3∶2.Ninety-three point eight percentage of MCL patients were in Ann Arbor stageⅢtoⅣ who had received more than one cycles of CHOP（E）±R chemotherapy.After analyze the clinical factors,we found that：①Respectively compared the three groups of MIPI5.7,Ki-67≤40%andβ2-GM3mg/L with the three groups of MIPI≥5.7,Ki-6740% andβ2-GM≥3mg/L,the short-term remission rates had no significant difference,while the 2years PFS of the previous three groups were longer than that of the posterior three groups（66.7%vs 25.0%,63.6%vs 19.1%,78.9%vs 33.5%,P〈0.05）.②SOX-11protein has been detected in nine cases and 66.7%of cases were positive without prognosis-associated relationship.③The complete remission rate and the 2years PFS of CHOP（E）＋R and PAD regimen were superior to those of the CHOP（E）regimen（57.1%vs 100%vs 0,71.4%vs 100%vs 11.1%,P〈0.05）.④Among three relapsed/refractory MCL patients,one who was treated with autologous peripheral blood stem cell transplantation and the other two who were received PAD regimen chemotherapy all had long-term PFS.Conclusion：Most patients with MCL were male and usually in Ann Arbor stageⅢtoⅣ.MIPI,Ki-67andβ2-GM play an important role in prediction of MCL prognosis.SOX-11in prognosis of MCL still needs to be made an intensive study.CHOP（E）＋R regimen is more effective than CHOP（E）.Curative effect of PAD regimen combined maintenance rituximab for refractory/relapse cases is distinct which remains to be further clinical observed.