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Br J Haematol. 2016 May;173(4):560-72. doi: 10.1111/bjh.13958. Epub 2016 Feb 23.

Anaplastic large cell lymphoma in paediatric and young adult patients.

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Department of Pathology, Division of Molecular Histopathology, University of Cambridge, Cambridge, UK.
European Research Initiative for ALK related Malignancies, Toulouse, France.
Institut Universitaire de Cancérologie Oncopole, 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.
Département de Cancérologie de l'Enfant et l'Adolescent, Gustave Roussy, Villejuif, France.


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.


ALK inhibitors; T cell lymphoma; anaplastic large cell lymphoma; anaplastic lymphoma kinase

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