Sézary syndrome with a complex, frameshift p53 gene mutation in a Chernobyl survivor.
We report a case of Sézary syndrome in a patient who was in the immediate vicinity of the Chernobyl nuclear reactor accident 18 months prior to presentation. A complex, frameshift p53 gene mutation was subsequently identified in tumour tissue, consisting of an 8-base pair deletion and a T-->G point mutation in exon 7. This is characteristic of damage caused by ionizing radiation, which suggests a causal link between exposure to ionizing radiation and the subsequent development of Sézary syndrome, a rare form of T-cell leukaemia/lymphoma.
Fraser-Andrews
E A
EA
Skin Tumour Unit, Department of Photobiology, St. John's Institute of Dermatology, St. Thomas' Hospital, London, UK. efraser-andrews@doctors.org.uk
McGregor
J M
JM
Crook
T
T
Brookes
L
L
Calonje
E
E
Whittaker
S J
SJ
D017353:Gene Deletion; D016158:Genes, p53; D006801:Humans; D008297:Male; D008875:Middle Aged; D017354:Point Mutation; D011210:Power Plants; D018788:Radioactive Hazard Release; D017422:Sequence Analysis, DNA; D012751:Sezary Syndrome / Q000209:etiology* / Q000235:genetics; D012878:Skin Neoplasms / Q000209:etiology* / Q000235:genetics; D014455:Ukraine