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儿童群体中的惰性淋巴瘤:滤泡性淋巴瘤、IRF4/MUM1+淋巴瘤、结内边缘区淋巴瘤和慢性淋巴细胞白血病。

Indolent lymphomas in the pediatric population: follicular lymphoma, IRF4/MUM1+ lymphoma, nodal marginal zone lymphoma and chronic lymphocytic leukemia.

作者信息

Quintanilla-Martinez Leticia, Sander Birgitta, Chan John K C, Xerri Luc, Ott German, Campo Elias, Swerdlow Steven H

机构信息

Institute of Pathology and Neuropathology, Eberhard Karls University of Tübingen and Comprehensive Cancer Center, University Hospital Tübingen, Liebermeisterstrasse 8, 72076, Tübingen, Germany.

Department of Laboratory Medicine, Division of Pathology, Karolinska Institut and Karolinska University Hospital Huddinge, Stockholm, Sweden.

出版信息

Virchows Arch. 2016 Feb;468(2):141-57. doi: 10.1007/s00428-015-1855-z. Epub 2015 Sep 28.

Abstract

Indolent lymphomas in the pediatric population were discussed during the 2014 European Association for Haematopathology/Society of Hematopathology workshop in Istanbul, Turkey. This session was focused on pediatric-type follicular lymphoma (FL), and its differential diagnosis with the newly recognized entity of IRF4/MUM1+ lymphomas mainly involving Waldeyer's ring. The differential diagnosis between t(14;18) negative FL grade 1/2 and pediatric-type FL in adults was highlighted. The overlapping pathological and clinical features between FL and nodal marginal zone lymphoma (NMZL) in children and young adults were recognized and morphologic and immunophenotypical criteria helpful for the differential diagnosis were presented. Both pediatric-type FL and NMZL are indolent processes that should be distinguished from atypical lymphoid hyperplasia of the tonsils and lymph nodes. The demonstration of a B cell monoclonal population by molecular studies is strongly recommended for the diagnosis. Recognition of these indolent variants to avoid overtreatment was emphasized. Whereas most indolent lymphomas in the pediatric population show characteristic clinical, pathologic, and genetic features that differ from the adult counterpart, other rare indolent lymphoid tumors such as chronic lymphocytic leukemia (CLL) have similar characteristics. In this report, novel findings, areas of special interest, and diagnostic challenges emerging from the cases submitted to the workshop will be discussed.

摘要

2014年欧洲血液病理学协会/血液病理学学会研讨会在土耳其伊斯坦布尔召开期间,对儿童群体中的惰性淋巴瘤进行了讨论。本次会议聚焦于儿童型滤泡性淋巴瘤(FL),以及它与新认识的主要累及瓦尔代尔环的IRF4/MUM1+淋巴瘤实体的鉴别诊断。强调了成人中t(14;18)阴性的1/2级FL与儿童型FL之间的鉴别诊断。认识到儿童和青年中FL与结内边缘区淋巴瘤(NMZL)在病理和临床特征上的重叠,并提出了有助于鉴别诊断的形态学和免疫表型标准。儿童型FL和NMZL均为惰性病程,应与扁桃体和淋巴结的非典型淋巴样增生相鉴别。强烈建议通过分子研究证实B细胞单克隆群体以进行诊断。强调了认识这些惰性变异型以避免过度治疗。虽然儿童群体中的大多数惰性淋巴瘤表现出与成人不同的特征性临床、病理和遗传学特征,但其他罕见的惰性淋巴样肿瘤,如慢性淋巴细胞白血病(CLL),具有相似的特征。在本报告中,将讨论提交至本次研讨会的病例中出现的新发现、特别感兴趣的领域以及诊断挑战。

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