ABSTRACT

Introduction 1354 Epidemiology of pediatric anaplastic large cell lymphoma 1354 Biology 1354 Anaplastic lymphoma kinase 1354 Functional activity of ALK in anaplastic large cell 1355

lymphoma cells Clinical presentation 1355 Diagnosis 1357 Staging 1358 Prognostic factors 1359

Treatment of anaplastic large cell lymphoma 1360 Clinical trials 1360 Radiation therapy 1362 Bone marrow transplantation 1362 Central nervous system disease and CNS prophylaxis 1362 Isolated skin lesions 1362 Treatment at recurrence 1363 Future directions 1363 Key points 1363 References 1364

Anaplastic large cell lymphoma (ALCL) as reported in the World Health Organization (WHO) classification1 was first described by Stein et al.2 in 1985 as a separate entity, defined by a frequently cohesive proliferation of large pleomorphic blasts and a constant expression of the CD30/ Ki-1 antigen on the tumor cells.3