Primary nodal marginal zone lymphomas of splenic and MALT type.
Primary nodal marginal zone lymphomas of splenic and MALT type.
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脾和 MALT 型原发性淋巴结边缘区淋巴瘤。
DOI：
10.1097/00000478-199901000-00006
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发表时间：
1999
期刊：
影响因子：
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通讯作者：
Jaffe,ES
中科院分区：
文献类型：
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作者：
Campo,E;Miquel,R;Krenacs,L;Sorbara,L;Raffeld,M;Jaffe,ES
原发性淋巴结边缘区淋巴瘤（MZL）的存在是有争议的，因为它们与假定的淋巴结对应物的关系也是如此。大多数伴有单核细胞样B细胞/边缘区分化的淋巴结淋巴瘤表现出淋巴结MALT淋巴瘤的形态学和免疫表型特征。脾边缘区淋巴瘤（SMZL）也是边缘区起源的，但其免疫表型与MALT淋巴瘤不同。为了阐明淋巴结和淋巴结MZL之间的关系，并研究SMZL淋巴结变异的可能存在，对36例最初被认为是原发性淋巴结肿瘤的MZL进行了检查。排除了其他伴有边缘区分化的低度淋巴瘤（小淋巴细胞性淋巴瘤/慢性淋巴细胞性白血病[SLL/CLL]、滤泡性淋巴瘤和套细胞淋巴瘤）。6个淋巴结MZL的形态和表型特征与SMZL相似，而30个肿瘤更类似于MALT型淋巴瘤。6例具有SMZL特征的肿瘤表现为残留生发中心周围的多形性浸润，缺乏或非常减弱的套囊。免疫球蛋白IgD阳性（6/6），而细胞周期蛋白D1（0/5）、CD 5（0/6）和CD 23（0/6）阴性。其中5名患者在I期或II期接受治疗。在诊断或随访期间，没有患者表现出脾肿大、外周血和/或骨髓浸润。30例具有MALT型特征的患者的淋巴结显示窦周和血管周围单核细胞样/中心细胞样细胞浸润和残留的生发中心，具有相对保存良好的套囊。肿瘤细胞IgD（0/17）、cyclin D1（0/8）和CD 5（0/12）均为阴性。16例有详细病史和临床随访的患者中有7例（44%）有淋巴结淋巴瘤的证据。这些观察结果表明，大多数淋巴结B细胞淋巴瘤与边缘区分化的MALT型，他们经常与一个淋巴结成分。此外，也存在脾MZL的原发淋巴结对应物，并且可以在没有脾肿大的情况下发生。
The existence of primary nodal marginal zone lymphomas (MZL) is controversial, as is their relationship to putative extranodal counterparts. Most nodal lymphomas with monocytoid B cell/marginal zone differentiation exhibit the morphologic and immunophenotypical characteristics of extranodal MALT-lymphomas. Splenic marginal zone lymphoma (SMZL) is also of putative marginal zone derivation, but it differs immunophenotypically from MALT lymphoma. To clarify the relationship between nodal and extranodal MZLs and to investigate the possible existence of a nodal variant of SMZL, 36 MZL initially considered to be primary nodal neoplasms were examined. Other low-grade lymphomas with marginal zone differentiation were excluded (small lymphocytic lymphoma/chronic lymphocytic leukemia [SLL/CLL], follicular lymphoma, and mantle cell lymphoma). Six nodal MZLs showed morphologic and phenotypic characteristics similar to those of SMZL, whereas 30 tumors were more similar to MALT-type lymphomas. The six tumors with SMZL features showed a polymorphic infiltrate surrounding residual germinal centers with absent or very attenuated mantle cuffs. These lymphomas were IgD positive (6/6) but cyclin D1 (0/5), CD5 (0/6), and CD23 (0/6) negative. Five of these patients came for treatment in stage I or II. No patient manifested splenomegaly, peripheral blood, and/or bone marrow infiltration either at diagnosis or during follow-up. Lymph nodes from 30 patients with MALT-type features showed a perisinusoidal and perivascular infiltration of monocytoid/centrocytoid cells and residual germinal centers with a relatively well-preserved mantle cuff. The neoplastic cells were negative for IgD (0/17), cyclin D1 (0/8), and CD5 (0/12). Seven of 16 (44%) patients with a detailed history and clinical follow-up had evidence of extranodal lymphoma. These observations suggest that most nodal B cell lymphomas with marginal zone differentiation are of the MALT type and that they are frequently associated with an extranodal component. In addition, a primary nodal counterpart of splenic MZL also exists, and may occur in the absence of splenomegaly.