伴显著边缘带分化的滤泡性淋巴瘤的临床病理学特征及预后
| 作者: |
1,2张丹丹,
3魏彦栓,
1张燕林,
1郑媛媛,
1谢建兰,
1周小鸽
1 首都医科大学附属北京友谊医院病理科，北京 100050 2 郑州大学第一附属医院病理科，郑州 454550 3 河南省第二人民医院检验科，郑州 451191 |
| 通讯: |
周小鸽
Email: zhouxiaoge59@hotmail.com |
| DOI: | 10.3978/j.issn.2095-6959.2020.10.006 |
摘要
目的：探讨一组形态学伴边缘带分化的滤泡性淋巴瘤(follicular lymphoma，FL)的临床特点、发病机制、诊断、鉴别诊断及预后。方法：收集北京友谊医院病理科2008年至2017年伴边缘带分化的FL患者12例，整理临床资料，观察形态学，进行免疫组织化学检测及分析，最后电话随访患者。结果：患者男5例，女7例，年龄30~77(中位年龄53)岁；11例临床表现为多发淋巴结病变，1例为多发淋巴结肿大伴脾脏多发结节；Ann Arbor分期I~II期及III~IV期各6例。镜下12例均以滤泡增生为主，8例为低级别，4例为高级别(3a级)；肿瘤性滤泡周围围绕明显的边缘带样细胞；边缘带样细胞胞质丰富淡染，细胞核小、不规则、扭曲，可见核皱褶，部分类似中心细胞的细胞核，可见散在中心母细胞。免疫组织化学：12例肿瘤细胞均表达B细胞抗原CD20，CD79α及Pax-5，不表达T细胞抗原CD3，CD5；生发中心细胞标志物(CD10及Bcl-6)在肿瘤性滤泡区(neoplastic follicular region，NFR)及边缘带分化区(marginal zone differentiation region，MZDR)的表达不一致，表现为：NFR，12例均弥漫表达；MZDR，12例均可见表达，但阳性细胞数量不等，其中10例(83.3%)散在、1例(8.3%)部分及1例(8.3%))较多细胞阳性；Bcl-2，11例弥漫阳性，1例(WHO 3a级)NFR阴性、MZDR阳性。随访时间1~113个月(中位数24个月)，7例疾病稳定，2例进展(1例转化为弥漫大B细胞淋巴瘤)，3例失访。结论：伴边缘带分化的FL形态特殊，生发中心标志物(CD10及Bcl-6)在FL的MZDR总是可以见到阳性细胞，这在其鉴别诊断中具有重要价值，也提示边缘带样细胞可能是FL的肿瘤成分。伴边缘带分化的FL可以向弥漫大B细胞淋巴瘤转化。
关键词：
滤泡性淋巴瘤；边缘带；单核样B细胞；免疫组织化学；预后
Clinicopathologic features and prognosis of follicular lymphoma with marginal zone B-cell differentiation
CorrespondingAuthor: ZHOU Xiaoge Email: zhouxiaoge59@hotmail.com
DOI: 10.3978/j.issn.2095-6959.2020.10.006
Abstract
Objective: To evaluate the clinical features, pathogenesis, differential diagnosis and prognosis of a series of Follicular lymphoma (FL) with marginal zone B-cell differentiation. Methods: Totally 12 cases of FL with marginal zone B-cell differentiation were collected from 2008 to 2017 in Beijing Friendship Hospital. The clinicopathological features, immunophenotype and follow-up data were analyzed. Results: There were 5 males and 7 females, ranged in age from 30 to 77 years (median, 53 years). All patients presented with multiple lymphadenopathies, except for 1 had additional splenic nodules, half of who were at I–II or III–IV in Ann Arbor stage. Morphologically, all cases showed follicular growth pattern, including 8 cases of low grade and 4 cases of high grade (grade 3a). The neoplastic follicles surrounded by marginal zone like-cells distinctly were seen in all cases. The marginal zone-like cells featured abundant pale cytoplasm and small, irregular, distorted or cleaved nuclei, partly resembling centrocyte-like cells, and immunoblasts were scattered. Immunohistochemically, the tumor cells were positive for CD20, CD79α and Pax-5, and negative for CD3, CD5. The expression of the germinal center markers (CD10 and Bcl-6) in the neoplastic follicular region (NFR) cells were different from that in the marginal zone differentiation region (MZDR) cells: in NFR, all 12 cases expressed diffusely; In MZDR, the distribution of the weakly positive (CD10)/positive (Bcl-6) cells varied featuring scattered (83.3%, n=10), focal (8.3%, n=1) and most (8.3%, n=1) staining. Bcl-2 was diffusely positive in all cases but 1 (grade 3a), in which cells were negative in NFR and positive in MZDR. The median follow-up time was 24 months (1–113 months). The follow-up was available in 9 out of 12 cases: 7 patients were stable, 2 progressed (1 case showed a transformation to diffuse large B cell lymphoma). Conclusion: FL with marginal zone differentiation is rare and distinct. Positive cells of germinal center markers (CD10, Bcl-6) are always found in MZDR of FL, which can contribute to the diagnosis and also indicates that marginal zone-like cells are not reactive but the neoplastic nature. FL with marginal zone B-cell differentiation can transform to diffuse large B cell lymphoma at times.
Keywords：
follicular lymphoma; marginal zone; monocytoid B cell; immunohistochemistry; prognosis