文章：

儿童T细胞和NK细胞淋巴瘤：新的生物学见解和治疗策略

Pediatric T- and NK-cell lymphomas: new biologic insights and treatment strategies

原文发布日期：2012-04-13

DOI: 10.1038/bcj.2012.8

类型: Review

开放获取: 是

英文摘要：

T- and natural killer (NK)-cell lymphomas are challenging childhood neoplasms. These cancers have varying presentations, vast molecular heterogeneity, and several are quite unusual in the West, creating diagnostic challenges. Over 20 distinct T- and NK-cell neoplasms are recognized by the 2008 World Health Organization classification, demonstrating the diversity and potential complexity of these cases. In pediatric populations, selection of optimal therapy poses an additional quandary, as most of these malignancies have not been studied in large randomized clinical trials. Despite their rarity, exciting molecular discoveries are yielding insights into these clinicopathologic entities, improving the accuracy of our diagnoses of these cancers, and expanding our ability to effectively treat them, including the use of new targeted therapies. Here, we summarize this fascinating group of lymphomas, with particular attention to the three most common subtypes: T-lymphoblastic lymphoma, anaplastic large cell lymphoma, and peripheral T-cell lymphoma-not otherwise specified. We highlight recent findings regarding their molecular etiologies, new biologic markers, and cutting-edge therapeutic strategies applied to this intriguing class of neoplasms.

摘要翻译： 

T细胞和自然杀伤（NK）细胞淋巴瘤是极具挑战性的儿童肿瘤。这类癌症临床表现多样，分子异质性显著，其中多种类型在西方相当罕见，造成诊断困难。2008年世界卫生组织分类已确认超过20种不同的T细胞和NK细胞肿瘤，可见该类疾病的高度多样性和潜在复杂性。在儿科人群中，由于大多数恶性肿瘤尚未经过大规模随机临床试验研究，最佳治疗方案的选择成为额外难题。尽管这类肿瘤罕见，但令人振奋的分子发现正不断深化我们对这些临床病理实体的认知，提高诊断准确性，并拓展治疗能力——包括新型靶向疗法的应用。本文针对这类引人关注的淋巴瘤进行综述，重点探讨三种最常见亚型：T淋巴母细胞淋巴瘤、间变性大细胞淋巴瘤及非特指型外周T细胞淋巴瘤，着重介绍其分子病因学新发现、新型生物学标志物以及用于这类特殊肿瘤的前沿治疗策略。

原文链接：

Pediatric T- and NK-cell lymphomas: new biologic insights and treatment strategies