Mature (non-anaplastic, non-cutaneous) T-/NK-cell lymphomas in children, adolescents and young adults: state of the science.

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 Haematol

authors

Flower A,Xavier AC,Cairo MS

doi

10.1111/bjh.15767

subject

Has Abstract

pub_date

2019-05-01 00:00:00

pages

418-435

issue

3

eissn

0007-1048

issn

1365-2141

journal_volume

185

pub_type

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