Class-switched Primary Cutaneous Marginal Zone Lymphomas Are Frequently IgG4-positive and Have Features Distinct From IgM-positive Cases

E. Carlsen, S. Swerdlow, J. Cook, Sarah E. Gibson
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引用次数: 27

Abstract

Primary cutaneous marginal zone lymphoma (PCMZL) can be subdivided into 2 groups based on immunoglobulin (Ig) heavy chain usage: IgM-positive cases that constitute a less common and more T-helper type 1–driven process, and more common heavy chain class-switched cases that are predominantly T-helper type 2–driven. Although some report a significant IgG4-positive subset, others have found a much smaller proportion. To further evaluate the proportion of IgG4-positive PCMZL, to address whether IgG4-positive cases have any distinctive characteristics, and to assess whether additional features separating IgM-positive and class-switched cases could be identified, the clinicopathologic features of 26 PCMZL obtained from 19 patients were investigated. Twenty of 26 (77%) PCMZL were heavy chain class-switched (19 IgG-positive, 1 IgA-positive), including 9 that were IgG4-positive (35%). IgG4-positive and other class-switched PCMZL were morphologically similar. IgM-positive cases occurred in older individuals (median: 69 vs. 46.5 y; P=0.0001), more often involved the subcutis (P=0.002), demonstrated plasma cells diffusely scattered versus at the periphery of the lymphoid infiltrate (P=0.005), uniformly showed follicular colonization (P=0.0001), contained more numerous B cells (P=0.0004), and were more likely to have a T-cell CD4:CD8 ratio of <3:1 (P=0.03). None of the IgM-positive PCMZL harbored a MYD88 L265P mutation. No significant differences in clinical outcome were documented. These results highlight the high frequency of IgG4-positive PCMZL, which are otherwise similar to other class-switched cases, provide additional evidence supporting the distinction between class-switched and IgM-positive cases, and emphasize the indolent nature of at least the class-switched PCMZL, which may warrant their categorization as a clonal chronic lymphoproliferative disorder.
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原发性皮肤边缘区淋巴瘤通常为igg4阳性,其特征与igm阳性病例不同
原发性皮肤边缘区淋巴瘤(PCMZL)可根据免疫球蛋白(Ig)重链的使用情况细分为两组:igm阳性病例构成较不常见的t辅助型1驱动过程,以及较常见的重链类型切换病例,主要是t辅助型2驱动。虽然有些报告了显著的igg4阳性亚群,但其他人发现的比例要小得多。为了进一步评估igg4阳性PCMZL的比例,解决igg4阳性病例是否具有任何独特的特征,并评估是否可以识别区分igm阳性和类别转换病例的其他特征,研究了19例患者的26例PCMZL的临床病理特征。26例PCMZL中有20例(77%)为重链切换型(igg阳性19例,iga阳性1例),其中igg4阳性9例(35%)。igg4阳性和其他类别切换的PCMZL在形态上相似。igm阳性病例发生在老年人中(中位数:69 vs 46.5;P=0.0001),更常累及皮下(P=0.002),浆细胞弥漫性分散而不是淋巴浸润周围(P=0.005),均匀地显示滤泡定植(P=0.0001),含有更多的B细胞(P=0.0004), t细胞CD4:CD8比<3:1的可能性更大(P=0.03)。igm阳性的PCMZL中没有MYD88 L265P突变。临床结果无显著差异。这些结果强调了igg4阳性PCMZL的高频率,这与其他类别转换病例相似,提供了额外的证据支持类别转换和igm阳性病例之间的区别,并强调了至少类别转换PCMZL的惰性性质,这可能证明了它们被归类为克隆性慢性淋巴细胞增生性疾病。
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