Peripheral T-cell lymphoma in childhood: population-based experience in the United Kingdom over 20 years.

摘要

BACKGROUND: Peripheral T-cell lymphomas (PTCL) are very rare in children and this has prevented assessment of best treatment and prognosis. PROCEDURE: 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 groupB (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.