title: Anaplastic large cell lymphoma in paediatric and young adult patients.
abstract: Anaplastic large cell lymphoma (ALCL) is a heterogeneous disease of debateable origin that, in children, is largely anaplastic lymphoma kinase (ALK) positive with aberrant ALK activity induced following the formation of chromosomal translocations. Whilst the survival rates for this disease are relatively high, a significant proportion (20-40%) of patients suffer disease relapse, in some cases on multiple occasions and therefore suffer the toxic side-effects of combination chemotherapy. Traditionally, patients are treated with a combination of agents although recent data from relapse patients have suggested that low risk patients might benefit from single agent vinblastine and, going forward, the addition of ALK inhibitors to the therapeutic regimen may have beneficial consequences. There are also a plethora of other drugs that might be advantageous to patients with ALCL and many of these have been identified through laboratory research although the decision as to which drugs to implement in trials will not be trivial.
authors.affiliation: Department of Pathology, Division of Molecular Histopathology, University of Cambridge, Cambridge, UK.|European Research Initiative for ALK related Malignancies, Toulouse, France.
authors.forename: Suzanne D
authors.initials: SD
authors.lastname: Turner
authors.affiliation: European Research Initiative for ALK related Malignancies, Toulouse, France.|Institut Universitaire de Cancérologie Oncopole, Toulouse, France.
authors.forename: Laurence
authors.initials: L
authors.lastname: Lamant
authors.affiliation: European Research Initiative for ALK related Malignancies, Toulouse, France.|Clinical Institute of Pathology, Medical University of Vienna, Vienna, Austria.|Ludwig Boltzmann Institute for Cancer Research (LBI-CR), Vienna, Austria.|Department of Laboratory Animal Pathology, University of Veterinary Medicine Vienna, Vienna, Austria.
authors.forename: Lukas
authors.initials: L
authors.lastname: Kenner
authors.affiliation: European Research Initiative for ALK related Malignancies, Toulouse, France.|Département de Cancérologie de l'Enfant et l'Adolescent, Gustave Roussy, Villejuif, France.
authors.forename: Laurence
authors.initials: L
authors.lastname: Brugières
mesh_terms: D000293:Adolescent; D000328:Adult; D000077548:Anaplastic Lymphoma Kinase; D000970:Antineoplastic Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D006801:Humans; D017728:Lymphoma, Large-Cell, Anaplastic / Q000175:diagnosis / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology; D008875:Middle Aged; D058990:Molecular Targeted Therapy; D020794:Receptor Protein-Tyrosine Kinases / Q000037:antagonists & inhibitors; D055815:Young Adult
chemical_list: D000970:Antineoplastic Agents; C000626173:ALK protein, human; D000077548:Anaplastic Lymphoma Kinase; D020794:Receptor Protein-Tyrosine Kinases
keywords: ALK inhibitors; T cell lymphoma; anaplastic large cell lymphoma; anaplastic lymphoma kinase