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结内单核细胞样B细胞淋巴瘤(结内边缘区B细胞淋巴瘤)

Nodal monocytoid B-cell lymphoma (nodal marginal-zone B-cell lymphoma).

作者信息

Nathwani B N, Drachenberg M R, Hernandez A M, Levine A M, Sheibani K

机构信息

Department of Hematopathology, University of Southern California, Los Angeles, USA.

出版信息

Semin Hematol. 1999 Apr;36(2):128-38.

PMID:10319381
Abstract

Benign monocytoid B cells are seen in lymph nodes in different types of lymphadenitis and they occur in the form of clusters within and around sinuses and in the interfollicular areas, but rarely completely surround benign follicles to produce a marginal-zone pattern. The cytologic hallmark of these cells is the presence of abundant pale to clear cytoplasm; these cells usually are of medium size, and they have a rather bland-appearing, irregular nuclei with inconspicuous nucleoli. Malignant monocytoid B-cell proliferations in a lymph node have been classified as monocytoid B-cell lymphomas (MBCL), which are now called nodal marginal-zone B-cell lymphoma (MZL) in the World Health Organization (WHO) classification. In the recently published clinical evaluation of the International Lymphoma Study Group classification of non-Hodgkin's lymphoma, 25 of 1,378 cases (1.8%) were classified as primary MZL, whereas four times as many cases (105 or 7.6%) were classified as low-grade mucosa-associated lymphoid tissue (MALT)-type lymphoma. Transformation to large-cell lymphoma at the time of diagnosis was seen in five of 25 (20%) cases of nodal MZL and in 32 of 105 (30%) cases of MALT-type lymphoma. Comparison of the clinical findings at presentation and the survival results indicate that nodal MZL is more aggressive clinically than low-grade MALT-type lymphoma. For example, patients with nodal MZL had a significantly higher incidence of advanced-stage disease, including peripheral and paraaortic lymphadenopathy, than those with MALT-type lymphoma. Moreover, patients with nodal MZL had lower 5-year overall survival and failure-free survival than patients with MALT type lymphoma. When analysis was restricted to those patients with zero to three adverse risk factors in the International Prognostic Index, patients with nodal MZL still had a significantly lower overall and failure-free survival at 5 years than patients with MALT-type lymphoma. We conclude that nodal MZL is a distinctive disease entity and is similar to other low-grade nodal lymphomas, such as the follicular or small lymphocytic lymphomas, but different than MALT-type lymphoma.

摘要

良性单核样B细胞可见于不同类型淋巴结炎的淋巴结中,它们以簇状形式出现在窦内、窦周及滤泡间区,但很少完全围绕良性滤泡形成边缘区模式。这些细胞的细胞学特征是含有丰富的淡染至清亮的胞质;这些细胞通常中等大小,核外观较为平淡、不规则,核仁不明显。淋巴结中的恶性单核样B细胞增殖已被归类为单核样B细胞淋巴瘤(MBCL),在世界卫生组织(WHO)分类中现称为结内边缘区B细胞淋巴瘤(MZL)。在最近发表的国际淋巴瘤研究组对非霍奇金淋巴瘤分类的临床评估中,1378例病例中有25例(1.8%)被归类为原发性MZL,而归类为低级别黏膜相关淋巴组织(MALT)型淋巴瘤的病例数是其4倍(105例或7.6%)。在25例结内MZL病例中有5例(20%)在诊断时转化为大细胞淋巴瘤,在105例MALT型淋巴瘤病例中有32例(30%)出现这种情况。对初诊时临床表现和生存结果的比较表明,结内MZL在临床上比低级别MALT型淋巴瘤更具侵袭性。例如,结内MZL患者晚期疾病的发生率显著高于MALT型淋巴瘤患者,包括外周和主动脉旁淋巴结病。此外,结内MZL患者的5年总生存率和无失败生存率低于MALT型淋巴瘤患者。当分析仅限于国际预后指数中具有零至三个不良风险因素的患者时,结内MZL患者的5年总生存率和无失败生存率仍显著低于MALT型淋巴瘤患者。我们得出结论,结内MZL是一种独特的疾病实体,与其他低级别结内淋巴瘤,如滤泡性或小淋巴细胞淋巴瘤相似,但与MALT型淋巴瘤不同。

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