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儿童和青少年间变性大细胞淋巴瘤

Anaplastic large cell lymphoma in children and adolescents.

作者信息

Lowe Eric J, Gross Thomas G

机构信息

Division of Pediatric Hematology-Oncology, Children's Hospital of the Kings Daughters, Norfolk, Virginia 25507, USA.

出版信息

Pediatr Hematol Oncol. 2013 Sep;30(6):509-19. doi: 10.3109/08880018.2013.805347. Epub 2013 Jun 12.

Abstract

Anaplastic Large Cell Lymphoma (ALCL) is the most common mature T-cell neoplasm in children and adolescents. ALCLs comprise approximately 15% of all non-Hodgkin lymphomas (NHL) in children and adolescents and commonly present with advanced systemic disease. Dissimilar from ALCL in adults, ALCL in children is nearly universally anaplastic large cell lymphoma kinase (ALK) positive. Despite the relative rarity of the disease, a great deal of insight into the pathogenesis of ALCL has been learned by researching the essential oncogenic role of ALK. Many different treatment strategies have been utilized with similar event free survival (EFS) rates of 65-75%. This review will provide an overview of the pathology, clinical features, prognostics factors, and treatment for children and adolescents with ALK positive ALCL.

摘要

间变性大细胞淋巴瘤(ALCL)是儿童和青少年中最常见的成熟T细胞肿瘤。ALCL约占儿童和青少年所有非霍奇金淋巴瘤(NHL)的15%,通常表现为晚期全身性疾病。与成人ALCL不同,儿童ALCL几乎普遍为间变性大细胞淋巴瘤激酶(ALK)阳性。尽管该疾病相对罕见,但通过研究ALK的关键致癌作用,人们对ALCL的发病机制有了深入了解。许多不同的治疗策略已被采用,无事件生存率(EFS)相似,为65%-75%。本综述将概述ALK阳性ALCL儿童和青少年的病理学、临床特征、预后因素及治疗方法。

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