Sézary syndrome with a complex, frameshift p53 gene mutation in a Chernobyl survivor.

Clin Exp Dermatol

Skin Tumour Unit, Department of Photobiology, St. John's Institute of Dermatology, St. Thomas' Hospital, London, UK.

Published: November 2001

AI Article Synopsis

  • A patient developed Sézary syndrome 18 months after being near the Chernobyl nuclear reactor accident.
  • Analysis of the tumor revealed a specific mutation in the p53 gene, which included an 8-base pair deletion and a point mutation linked to ionizing radiation damage.
  • The findings indicate a possible connection between ionizing radiation exposure from the Chernobyl incident and the onset of this rare T-cell leukemia/lymphoma.

Article Abstract

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.

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Source
http://dx.doi.org/10.1046/j.1365-2230.2001.00919.xDOI Listing

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