Four radiation hypersensitivity cases and their implications for clinical radiotherapy.

摘要: Abstract Background and purpose : Over a 20 year period, four out of 2000 paediatric radiotherapy patients, treated at St. Bartholomew's Hospital (three with lymphoma, one angiosarcoma), have revealed extreme/fatal clinical hypersensitivity in normal tissues. Patients methods Cellular was confirmed vitro attributed to the ataxia-telangiectasia (A-T) gene cases I II, newly described defect DNA ligase 4 case III, novel as yet incompletely defined, molecular IV who presented xeroderma pigmentosum (XP). Results The severe preceded cellular analysis, but did not manifest clinically exaggerated tissue reaction until 3+ weeks after start conventionally fractionated course radiotherapy, by which time latent damage had been inflicted. There were no stigmata alert clinician predisposing syndrome two patients (cases II). We point that approximately 20% A-T are classified variants delayed expression symptoms, II falls into this category. Conclusions As lymphoma (incidence, 100 000 children) constituted majority diagnoses, questions arise to: (1), probability other centres having experienced being future similar problems (particularly bearing mind oncologically radiosensitivity syndromes represented our experience); (2), appropriateness, efficiency applicability predictive assays. Unambiguous would apparent from clonal assays on fibroblast cultures all prior treatment, such take 4–6 produce results. While estimates chromosome or pre-treatment blood derived cells be faster, there is health economics issue general ‘screening'