[Clinical and biological features and prognosis of patients with leukemic non-nodal mantle cell lymphoma].

Objective: To investigate the clinical, biological and prognostic characteristics of leukemic non-nodal mantle cell lymphoma (nnMCL). Methods: The clinical data of 14 patients with nnMCL and 238 patients with classical mantle cell lymphoma (cMCL) in Blood Diseases Hospital, Chinese Academy of Medical Sciences from November 2000 to October 2020 were retrospectively analyzed. Among the 14 patients with nnMCL, there were 9 males and 5 females, with the age [M (Q1, Q3)] of 57.5 (52.3, 67.0) years. Among the 238 patients with cMCL, there were 187 males and 51 females, with the age of 58.0 (51.0, 65.3) years. The clinical and biological characteristics of the two groups were recorded and compared. Follow-up and efficacy evaluation were conducted by re-examination during hospital stay and telephone follow-up and so on. Results: The proportion of CD200 expression in nnMCL patients was 8/14, which was higher than that in cMCL patients [14.6% (19/130)] (P=0.001). The proportion of CD23 expression in nnMCL patients was 8/14, which was higher than that in cMCL patients [13.5% (23/171)] (P<0.001). The proportion of CD5 expression in nnMCL patients was 10/14, which was lower than that in cMCL patients [97.4% (184/189)] (P=0.001). The proportion of CD38 expression in nnMCL patients was 4/14, which was lower than that in cMCL patients [69.6% (112/161)] (P=0.005). The expression proportion of sex-determining region of Y chromosome-related high-mobility-group box 11 (SOX11) in nnMCL patients was 1/5, which was lower than that in cMCL patients [77.9% (60/77)] (P=0.014). The proportion of immunoglobulin heavy chain variable region (IGHV) mutations in nnMCL patients was 11/11, which was higher than that in cMCL patients [26.0% (13/50)] (P<0.001). As of April 11, 2021, the follow-up time for nnMCL and cMCL patients was 31 (8-89) months and 48 (0-195) months, respectively. Among the 14 nnMCL patients, 6 patients were still under observation, and 8 patients were treated. The overall response rate (ORR) was 8/8, including 4 patients with complete remission and 4 patients with partial response. The median overall survival and median progression-free survival were not reached in nnMCL patients. In the cMCL group, 50.0% (112/224) patients achieved a complete response, 24.6% (55/224) patients achieved a partial response, and ORR was 74.6% (167/224). There was no statistically significant difference in ORR between the two groups (P=0.205). Conclusions: nnMCL patients have an indolent progression, with higher expression rates of CD23 and CD200 and lower expression rates of SOX11, CD5 and CD38. Most patients have IGHV mutations, with a relatively good prognosis, and"watch and wait"approach is an optional treatment.目的： 分析白血病性非淋巴结型套细胞淋巴瘤（nnMCL）患者的临床和生物学特征以及预后特点。 方法： 回顾性分析2000年11月至2020年10月中国医学科学院血液病医院确诊的14例nnMCL患者和238例经典型套细胞淋巴瘤（cMCL）患者的临床资料。14例nnMCL患者中，男9例，女5例，年龄M（Q1，Q3）为57.5（52.3，67.0）岁；238例cMCL患者中，男187例，女51例，年龄为58.0（51.0，65.3）岁。记录并比较两组患者的临床和生物学特征，采取住院复查、电话随访等方式进行随访及疗效评估。 结果： nnMCL患者免疫表型CD200表达比例为8/14，高于cMCL患者的14.6%（19/130）（P=0.001）；nnMCL患者CD23表达比例为8/14，高于cMCL患者的13.5%（23/171）（P<0.001）；nnMCL患者CD5表达比例为10/14，低于cMCL患者的97.4%（184/189）（P=0.001）；nnMCL患者CD38表达比例为4/14，低于cMCL患者的69.6%（112/161）（P=0.005）；nnMCL患者Y染色体性别决定区相关高速泳动族框因子11（SOX11）表达比例为1/5，低于cMCL患者的77.9%（60/77）（P=0.014）；nnMCL患者免疫球蛋白重链可变区（IGHV）突变比例为11/11，高于cMCL患者的26.0%（13/50）（P<0.001）。截至2021年4月11日，nnMCL患者随访时间为31（8~89）个月，cMCL患者随访时间为48（0~195）个月。14例nnMCL患者中，6例仍在观察，8例开始治疗，总有效率（ORR）为8/8，完全缓解4例，部分缓解4例，中位总生存期、无进展生存期均未达到。cMCL组50.0%（112/224）患者获得完全缓解，24.6%（55/224）患者获得部分缓解，ORR为74.6%（167/224），两组ORR差异无统计学意义（P=0.205）。 结论： nnMCL患者呈惰性进程，CD23、CD200表达比例更高，SOX11、CD5、CD38表达比例更低，多数患者存在IGHV突变，预后相对较好，观察和等待是一个可选治疗方案。.