Abstract:
:Mature (non-anaplastic) T-cell and natural killer (NK)-lymphomas rarely occur in children or adolescents. Due to the low incidence and heterogeneity, information regarding the aetiology, physiopathology and genetics of paediatric mature (non-anaplastic) T/NK-cell lymphoma is lacking. In addition, standard treatments have not yet been established. In the absence of randomised clinical trials, anthracycline-containing regimens are usually considered as the first treatment option, but with discouraging outcomes, especially in patients with advanced disease. The implementation of autologous or allogeneic stem cell transplantation as upfront consolidation therapy or for chemotherapy-sensitive relapsed disease have resulted in improved survival for some patient subsets. The recent use of novel targeted molecular and immunotherapeutic agents has also been shown to be promising in small numbers of patients. In this context, we will review the current state of the scientific knowledge on the most common mature (non-anaplastic, non-cutaneous) T/NK-cell lymphomas occurring in children, adolescent and young adults.
journal_name
Br J Haematoljournal_title
British journal of haematologyauthors
Flower A,Xavier AC,Cairo MSdoi
10.1111/bjh.15767subject
Has Abstractpub_date
2019-05-01 00:00:00pages
418-435issue
3eissn
0007-1048issn
1365-2141journal_volume
185pub_type
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