间变性大细胞淋巴瘤
医学
间变性淋巴瘤激酶
淋巴瘤
疾病
肿瘤科
内科学
病理
癌症研究
恶性胸腔积液
肺癌
作者
Eric Lowe,Thomas G. Gross
标识
DOI:10.3109/08880018.2013.805347
摘要
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.
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