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[儿童和青少年恶性淋巴瘤。诊断实用知识]

[Malignant lymphomas in children and adolescents. Practical knowledge for diagnosis].

作者信息

Oschlies I, Klapper W

机构信息

Institut für Pathologie, Sektion Hämatopathologie und Lymphknotenregister, Christian-Albrecht-Universität Kiel, Universitätsklinikum Schleswig-Holstein, Campus Kiel, Arnold-Heller-Str. 3, Haus 14, 24105, Kiel, Deutschland,

出版信息

Pathologe. 2014 Jul;35(4):383-95; quiz 396-7. doi: 10.1007/s00292-014-1891-5.

Abstract

Malignant lymphomas are very rare in childhood. Therefore in Germany and Europe-wide the diagnosis and treatment of these diseases and research into them are carried out in multicenter-study groups and registries that provide central clinical consulting as well as histopathological, genetic, and molecular diagnoses. Despite these central structures, the diagnosis of these aggressive lymphomas is usually made initially outside these specialized centers. Therefore, in clinical situations that are often quite critical, every pathologist may be required to make the initial diagnosis and decide on the initial therapy regimen. Specific features related to the incidence, biology, and clinical presentation of pediatric lymphomas strongly influence the diagnostic workup and require a different diagnostic procedure from that employed in adults. In this paper, we will try to provide the most important information required for a tissue-saving initial diagnosis of the most common pediatric lymphomas. The proposed procedures allow reliable differentiation into the main lymphoma categories and, thus, provide the necessary information for the first therapeutic regimens, although the final subtyping will remain the responsibility of specialists. The most relevant histopathological features, appropriate immunohistochemical stains, and diagnostic pitfalls are demonstrated. As examples of the most common entities, special attention is paid to Burkitt lymphoma, diffuse large B-cell lymphoma, precursor T- and B-cell lymphoma, and anaplastic large-cell lymphoma.

摘要

恶性淋巴瘤在儿童时期非常罕见。因此,在德国乃至整个欧洲,这些疾病的诊断、治疗及相关研究是在多中心研究组和登记处开展的,这些机构提供中央临床咨询以及组织病理学、遗传学和分子诊断。尽管有这些中央机构,但这些侵袭性淋巴瘤的诊断通常最初是在这些专业中心之外做出的。因此,在往往相当危急的临床情况下,每个病理学家可能都需要做出初步诊断并决定初始治疗方案。与儿童淋巴瘤的发病率、生物学特性和临床表现相关的特定特征强烈影响诊断检查,并且需要采用与成人不同的诊断程序。在本文中,我们将尝试提供对最常见儿童淋巴瘤进行组织保存型初步诊断所需的最重要信息。所提议的程序能够可靠地将淋巴瘤分为主要类别,从而为首个治疗方案提供必要信息,不过最终的亚型分类仍由专家负责。文中展示了最相关的组织病理学特征、合适的免疫组化染色方法以及诊断陷阱。作为最常见实体的示例,特别关注了伯基特淋巴瘤、弥漫性大B细胞淋巴瘤、前体T细胞和B细胞淋巴瘤以及间变性大细胞淋巴瘤。

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