We found a match
Your institution may have rights to this item. Sign in to continue.
- Title
From bench to bedside: the past, present and future of therapy for systemic paediatric ALCL, ALK+.
- Authors
Larose, Hugo; Burke, G. A. Amos; Lowe, Eric J.; Turner, Suzanne D.
- Abstract
Summary: Anaplastic large cell lymphoma (ALCL) is a T cell Non‐Hodgkin Lymphoma that mainly presents in paediatric and young adult patients. The majority of cases express a chimeric fusion protein resulting in hyperactivation of anaplastic lymphoma kinase (ALK) as the consequence of a chromosomal translocation. Rarer cases lack expression of ALK fusion proteins and are categorised as ALCL, ALK−. An adapted regimen of an historic chemotherapy backbone is still used to this day, yielding overall survival (OS) of over 90% but with event‐free survival (EFS) at an unacceptable 70%, improving little over the past 30 years. It is clear that continued adaption of current therapies will probably not improve these statistics and, for progress to be made, integration of biology with the design and implementation of future clinical trials is required. Indeed, advances in our understanding of the biology of ALCL are outstripping our ability to clinically translate them; laboratory‐based research has highlighted a plethora of potential therapeutic targets but, with high survival rates combined with a scarcity of funding and patients to implement paediatric trials of novel agents, progress is slow. However, advances must be made to reduce the side‐effects of intensive chemotherapy regimens whilst maintaining, if not improving, OS and EFS.
- Subjects
ANAPLASTIC lymphoma kinase; CHIMERIC proteins; CHROMOSOMAL translocation; YOUNG adults; T cells
- Publication
British Journal of Haematology, 2019, Vol 185, Issue 6, p1043
- ISSN
0007-1048
- Publication type
Article
- DOI
10.1111/bjh.15763