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Sézary syndrome with a complex, frameshift p53 gene mutation in a Chernobyl survivor
Author(s) -
FraserAndrews E. A.,
McGregor J. M.,
Crook T.,
Brookes L.,
Calonje E.,
Whittaker S. J.
Publication year - 2001
Publication title -
clinical and experimental dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.587
H-Index - 78
eISSN - 1365-2230
pISSN - 0307-6938
DOI - 10.1046/j.1365-2230.2001.00919.x
Subject(s) - frameshift mutation , mutation , medicine , gene , genetics , cancer research , biology
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.