R. Elzein et al., CHROMOSOMAL RADIOSENSITIVITY OF LYMPHOCYTES FROM SKIN CANCER-PRONE PATIENTS, Mutation research. Section on environmental mutagenesis and related subjects, 335(2), 1995, pp. 143-149
Frequencies of spontaneous and radiation-induced chromosome aberration
s were documented in lymphocytes from patients with basal cell nevus s
yndrome (BCNS) and from those with epidermodysplasia verruciformis (EV
). Cells were irradiated with single or double doses of gamma-rays or
UV light. For the double dose irradiation protocol, the two doses were
separated from each other by 60 min. After irradiation of cells with
4 or 2 + 2 J/m(2) UV light, lymphocytes from seven BCNS patients had a
similar number of aberrant cells compared with normal controls, while
cells from three EV patients showed a significantly increased number
of cells containing aberrations, particularly chromatid-type aberratio
ns, compared to controls (p < 0.03, Student's t-test). Similar results
were observed whether the cells were irradiated with single or double
doses of UV light. In addition, lymphocytes from EV patients had sign
ificantly lower mitotic indices than controls whether the cultures wer
e irradiated with UV light or unirradiated (p < 0.05; Student's t-test
). After irradiation with 100, 100 + 100 or 50 + 50 cGy gamma-rays, th
e mean chromosome aberration frequencies from nine BCNS patients and t
wo EV patients were not significantly different from the controls. The
spontaneous chromosome aberration frequencies were similar for the pa
tients and the controls. Our data indicate that BCNS and EV lymphocyte
s irradiated with gamma-rays have a normal DNA repair response. Howeve
r, cells from EV patients have an abnormal repair response to UV light
induced DNA damage. This abnormality is probably caused by deficiency
in DNA repair and it is consistent with the patients' sensitivity to
UV light induced cutaneous squamous cell carcinomas.