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

Anaplastic large cell lymphoma in children and adolescents

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Anaplastic large cell lymphoma in children and adolescents

Eric J Lowe et al. Pediatr Hematol Oncol. 2013 Sep.

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.

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