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儿童滤泡性淋巴瘤、边缘区淋巴瘤和边缘区增生。

Pediatric follicular lymphomas, marginal zone lymphomas, and marginal zone hyperplasia.

作者信息

Swerdlow Steven H

机构信息

Department of Pathology, University of Pittsburgh School of Medicine, PA 15213, USA.

出版信息

Am J Clin Pathol. 2004 Dec;122 Suppl:S98-109. doi: 10.1309/4BKNAKE4D7CT3C1B.

DOI:10.1309/4BKNAKE4D7CT3C1B
PMID:15690646
Abstract

Whereas most B-cell lymphomas in children are of diffuse large B-cell or Burkitt type, other B-cell lymphomas, including follicular and marginal zone B-cell lymphomas, rarely occur Pediatric follicular lymphomas (FLs) have many features indistinguishable from those seen in adults but demonstrate an increased proportion that are localized, lack bcl-2 protein expression, lack bcl-2 translocations, and are grade 3 but still indolent. They also tend to have large expansile follicles. Although usually at nodal or tonsillar sites, other extranodal involvement also occurs, with FL of the testis well described. Distinction from potentially clonal but reactive follicular hyperplasia is important. Marginal zone B-cell lymphomas of nodal and extranodal types are other indolent B-cell neoplasms that usually are seen in adults but also occur in children. The differential diagnosis must include the very recently reported lambda light chain class-restricted marginal zone hyperplasias of the tonsil or appendix that might closely mimic a lymphoma. Understanding the full spectrum of B-cell neoplasia in children and its unique features is important for diagnostic, therapeutic, and academic purposes.

摘要

虽然儿童中的大多数B细胞淋巴瘤为弥漫性大B细胞型或伯基特型,但其他B细胞淋巴瘤,包括滤泡性和边缘区B细胞淋巴瘤,很少发生。儿童滤泡性淋巴瘤(FL)具有许多与成人所见难以区分的特征,但显示出局限性比例增加、缺乏bcl-2蛋白表达、缺乏bcl-2易位,且为3级但仍呈惰性。它们还往往有大的扩张性滤泡。虽然通常位于淋巴结或扁桃体部位,但也会出现其他结外受累情况,睾丸FL已有详细描述。与潜在的克隆性但反应性滤泡增生相鉴别很重要。淋巴结型和结外型边缘区B细胞淋巴瘤是其他惰性B细胞肿瘤,通常见于成人,但也发生于儿童。鉴别诊断必须包括最近报道的扁桃体或阑尾的λ轻链类别受限的边缘区增生,其可能与淋巴瘤极为相似。了解儿童B细胞肿瘤的全貌及其独特特征对于诊断、治疗和学术目的都很重要。

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