侵袭性变异型套细胞淋巴瘤临床病理特征及预后分析

目的探讨套细胞淋巴瘤侵袭性变异型(aggressive variants of mantle cell lymphoma,AV-MCL)的临床病理学特征及预后。方法回顾性分析72例AV-MCL和同期60例经典型套细胞淋巴瘤(mantle cell lymphoma,MCL)患者的临床资料,观察AV-MCL的组织学形态及免疫表型,应用FISH法检测Ig H/CCND1融合基因,并比较两组患者的临床病理特征及生存率差异。结果 72例AV-MCL占同期(466例) MCL的15. 4%,其中母细胞变异型占12. 0%,多形性变异型占3. 4%。男女比为2. 8∶1,中位年龄62. 0岁(范围34～86岁)。有B症状者占26. 3%(10/38)。Ann Arbor分期显示Ⅲ+Ⅳ期占64. 9%(24/37)。肿瘤组织由增生的异型淋巴细胞构成,56例(77. 8%)为母细胞变异型,16例(22. 2%)为多形性变异型。肿瘤细胞均表达CD20和Cyclin D1,CD5、SOX11、CD43、MUM1的表达比例分别为95. 2%(60/63)、62. 5%(10/16)、66. 7%(6/9)和51. 4%(19/37),CD10和BCL-6异常表达的比例分别为15. 6%(10/64)和16. 1%(9/56)。AV-MCL的Ki-67增殖指数显著高于经典型MCL(60. 1%±11. 7%vs 22. 7%±11. 0%,P <0. 001)。3例CD5阴性病例FISH检测结果阳性。25例(34. 7%)获得随访,中位随访时间20个月(4～60个月)。与经典型MCL相比,AV-MCL的总生存率及对应Ⅰ+Ⅱ期和Ⅲ+Ⅳ期总生存率差异均有统计学意义(P <0. 05)。单因素分析结果显示,在AV-MCL中伴结外侵犯的患者总生存率更低(P=0. 032)。结论 AV-MCL临床较罕见,大多为母细胞变异型,预后较经典型MCL差,伴结外侵犯是其不良预后因素。

Clinicopathological features and prognosis of aggressive variants of mantle cell lymphoma

Purpose To investigate the clinicopathological features and prognosis of aggressive variants (blastoid and pleomorphic) of mantle cell lymphoma (AV-MCL). Methods The clinical data of 72 cases of AV-MCL and 60 cases of common form of MCL (C-MCL) in the same period diagnosed were analyzed retrospectively. The histological morphology and immunophenotype of AV-MCL were observed. The clinicopathological characteristics and overall survival rates (OSRs) of two groups were compared. Results Seventy-two cases of AV-MCL accounted for 15.4% of MCL in the same period (466 cases),12.0% of blastoid and 3.4% of pleomorphic. The median age was 62 years (range :34~86 years,M ∶ F=2.8 ∶ 1.0). Those patients with B symptoms accounted for 26.3%(10/38). The Ann Arbor stage Ⅲ+Ⅳ accounted for 64.9%(24/37). The tumor tissue was made up of proliferating lymphoid cells. Fifty-six cases (77.8%) were blastoid,16 cases (22.2%) were pleomorphic. All tumors were positive for CD20 and Cyclin D1. The positive expression rates of CD5,SOX11,CD43 and MUM1 in the tumors were 95.2%(60/63),62.5%(10/16),66.7%(6/9) and 51.4%(19/37) respectively. The proportion of aberrant expression of CD10 and BCL-6 in tumor was 15.6%(10/64) and 16.1%(9/56),respectively. The AV-MCL Ki-67 marker index was significantly higher than that of C-MCL (60.1%±11.7% vs 22.7%±11.0%, P <0.001). FISH test was positive in 3 cases of CD5 negative. Twenty-five (34.7%) patients had a median follow-up time of 20.0 months (range :4-60 months). Compared with the C-MCL,the OSRs of the patients with AV-MCL,and the corresponding differences in the OSRs of stages Ⅰ+Ⅱ and Ⅲ+Ⅳ were statistically significant ( P <0.05). Univariate analysis showed that the OSRs was lower in AV-MCL patients with extranodal invasion ( P =0.032). Conclusions AV-MCL is rare,most of which are blastoid variant,and the prognosis is worse than that of C-MCL. Extranodal invasion is a bad prognostic factor.