Lead investigator: Dr Chris Bacon, Newcastle University
Funded by The Little Princess Trust and administered by CCLG
Funded July 2017
Award: £14,145.44
Children born with poorly functioning immune systems have a substantial additional risk of developing cancer, most commonly lymphoma - a varied group of tumours arising from lymphocytes (white blood cells in the lymphatic system).
These children often struggle to tolerate standard lymphoma treatment and have a high chance of dying from either therapy-related toxicity or recurrence of their cancer. However, such cases are uncommon and systematic collection and reporting of data relating to diagnosis, treatment and outcome is poor, leading to difficulties in making clinical management decisions.
We have developed a collaboration between the UK’s two commissioned paediatric immune deficiency bone marrow transplant units to analyse and describe the treatment strategies and outcomes of UK cases of lymphoma developing in children with immune deficiency.
Lymphomas in immune deficient patients are thought to arise as a result of complex interactions between the abnormal immune system, viral infection and genetic changes in developing lymphoma cells. However, the details of this are very poorly understood. As a result of this and the other co-existing conditions these patients often have, the accurate diagnosis and classification of lymphoma in these children is often extremely challenging and subject to variation.
The aim of this project is to perform a detailed, expert analysis of the lymphomas we have identified, using up-to-date techniques. This analysis will provide initial information on the types of lymphoma occurring in children with immune deficiency and will allow meaningful interpretation of the clinical data being collected.
However, this study is also a pilot project which will guide the patient selection, workflow and diagnostic approach of a much larger Europe-wide study of these rare cases being developed by three collaborating European childhood cancer and immunology organisations. Our cases will form the core UK contribution to the European study, which will be a powerful provider of information guiding recommendations for diagnosis and treatment. Importantly, the European study will also involve detailed genetic analysis of these lymphomas, paving the way for the development of newer, less toxic therapies for this vulnerable group of children.