淋巴结边缘区B细胞淋巴瘤的临床病理特征

目的探讨原发性淋巴结边缘区B细胞淋巴瘤（NMZL）的形态特点、免疫表型特征、鉴别诊断及预后。方法对10例原发于淋巴结的NMZL进行光镜观察,免疫组织化学EliVision法染色,参照Ann Arbor临床分期,并进行了3～29个月随访。结果10例就诊时均精神状况良好,但大多（6／7）临床分期较高（Ⅱ或Ⅲ期）。NMZL大多呈模糊结节的生长方式（5／10）。细胞的形态以中心细胞样细胞为主（7／10）,少数以单核细胞样细胞为主（2／10）或小淋巴细胞样细胞为主（1／10）。大多（8／10）伴有浆样细胞和（或）浆细胞。肿瘤细胞增殖指数范围5％～50％。7例滤泡树突细胞（FDC）网以萎缩变小为主,3例FDC网不同程度增生。7例得到随访（平均12个月）,其中6例临床分期为Ⅱ或Ⅲ期。3例存活1年以上。结论原发性NMZL少见,肿瘤细胞生长方式独特,细胞形态多以生发中心细胞样细胞为主。需与淋巴浆细胞淋巴瘤、结外边缘区淋巴瘤累及淋巴结及T区增生等鉴别。肿瘤细胞较易播散,诊断时大多临床分期较高,预后可能较差。

Nodal marginal zone B-cell lymphoma: a clinicopathologic study of 10 cases

OBJECTIVE: To study the morphologic features, immunophenotype, differential diagnosis and prognosis of nodal marginal zone B-cell lymphoma (NMZL). METHODS: Light microscopic examination and immunohistochemical study were carried out in 10 cases of NMZL. Seven of which had follow-up information available. RESULTS: All cases presented with good performance status at the time of diagnosis. Amongst the 7 cases with follow-up information available, most (6/7) were in advanced clinical stage (stage II to III). The one-year survival rate was 67%. A vaguely nodular growth pattern was observed in most cases of NMZL (5/10). The lymphoma was composed predominantly of atypical lymphoid cells resembling centrocytes (7/10). A predominance of monocytoid B-cell (2/10) or small lymphocytic (1/10) morphology was rare. Instead, the presence of a minor component of monocytoid B cells was not uncommon (5/10). Plasmacytoid or plasmacytic cells were also frequently found (8/10). The proliferation index ranged from 5% to 50%. Follicular dendritic cells appeared atrophic in 7 cases and variably hyperplasic in 3 cases. CONCLUSIONS: Primary NMZL is rare. It has a unique growth pattern and most cases are composed predominantly of cells resembling centrocytes. Differential diagnosis includes lymphoplasmacytic lymphoma, lymph node involvement by extranodal marginal zone B-cell lymphoma and T-zone hyperplasia. The clinical stage is often high at presentation, with systemic dissemination. The prognosis of NMZL is thus relatively poor.