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Br J Haematol. 2019 May;185(3):418-435. doi: 10.1111/bjh.15767. Epub 2019 Feb 1.

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

Flower A1,2, Xavier AC3, Cairo MS1,2,4,5,6.

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Department of Pediatrics, New York Medical College, Valhalla, NY, USA.
Department of Microbiology & Immunology, New York Medical College, Valhalla, NY, USA.
Division of Hematology/Oncology, Department of Pediatrics, Children's of Alabama/University of Alabama at Birmingham, Birmingham, AL, USA.
Department of Medicine, New York Medical College, Valhalla, NY, USA.
Department of Pathology, New York Medical College, Valhalla, NY, USA.
Department of Cell Biology & Anatomy, New York Medical College, Valhalla, NY, USA.


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.


extranodal NK-T-cell lymphoma; non-Hodgkin lymphoma; paediatrics; peripheral T-cell lymphoma; stem cell transplantation


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