Peripheral T-cell lymphoma in childhood: population-based experience in the United Kingdom over 20 years.
BACKGROUND
Peripheral T-cell lymphomas (PTCL) are very rare in children and this has prevented assessment of best treatment and prognosis.


METHODS
Registry-based experience in England, Scotland and Wales over a 20-year period was studied to address these shortfalls. Anaplastic large cell lymphoma and mycosis fungoides were excluded due to recent publications describing UK experience with these disorders.


RESULTS
Twenty-five cases were identified, comprising 1.6% of non-Hodgkin lymphoma (NHL) registrations; 17 (68%) children with PTCL-unspecified (PTCL-u), 3 (12%) with angiocentric PTCL, 3 (12%) with angioimmunoblastic PTCL, and 2 (8%) with subcutaneous panniculitis-like T-cell lymphoma. Eighteen children were male, with a male/female ratio of 2.6:1. Median age was 7 (range 1-14) years. Eleven children (44%) died and actuarial survival was 76% at 1 year, 64% at 3 years and 59% at 5 years. Treatments given were subdivided between group T (regimens for T NHL or acute lymphoblastic leukaemia) or group B (regimens for B NHL). Amongst the 17 children with PTCL-u, 9/12 children in group T survived compared with 1/5 group B.


CONCLUSIONS
The Children's Cancer and Leukaemia Group recommendation that children with PTCL-u receive group T therapy is supported by this series. The numbers of children with other types of PTCL were too small to allow conclusions on best therapy.
Windsor
Rachael
R
Department of Haematology and Oncology, Great Ormond Street Hospital for Children, London, United Kingdom.
Stiller
Charles
C
Webb
David
D
D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D016411:Lymphoma, T-Cell, Peripheral / Q000188:drug therapy* / Q000453:epidemiology / Q000473:pathology; D008297:Male; D012042:Registries; D016896:Treatment Outcome; D006113:United Kingdom / Q000453:epidemiology