Radiosensitivity

Radiation therapists have observed that when AT patients with cancer are treated with conventional doses of ionizing radiation, they develop sequelae characteristic of much higher dosages. The radiosensitivity can be demonstrated in vitro as well, usually

Cancer susceptlülllty

Atflülfl

-Cerebellar {feveSopment

-Purkinje cell depletion

LyTphoma-'leu^em h CintM wiln brent I can»'

P ul m onary in Teci la n s

'Jrnndu no tief œncies lgG2.IçA. IgET oelk ■Thymus «Jcvolopmum

Cancer susceptlülllty

LyTphoma-'leu^em h CintM wiln brent I can»'

P ul m onary in Teci la n s

'Jrnndu no tief œncies lgG2.IçA. IgET oelk ■Thymus «Jcvolopmum

Radiosensitivity

-Seifsne reaHkms -ftfldionesislarn OHA synthcss -Cotony survival

Chromosoms I ehs ngcs CelEcycl? c ncc k pa i nis

Copyright © 1998 Elsevier Ltd. All Rights Reserved. Published by Elsevier Ltd.

-t(7:14) translocations -Teiomeif lusiorti

■GvS forflsiiorioriirtg -Û.WI block

Radiosensitivity

-Seifsne reaHkms -ftfldionesislarn OHA synthcss -Cotony survival

Chromosoms I ehs ngcs CelEcycl? c ncc k pa i nis

Copyright © 1998 Elsevier Ltd. All Rights Reserved. Published by Elsevier Ltd.

-t(7:14) translocations -Teiomeif lusiorti

Figure 1 The AT syndrome.

by 'radioresistant DNA synthesis' or by colony survival efficiency following radiation. In the laboratory, carriers are intermediate in radiosensitivity between homozygotes and normals. AT cells are sensitive not only to ionizing radiation but to a variety of radiomimetic agents as well. Testing for radiation hypersensitivity can be used to confirm the diagnosis; however, few laboratories test clinical specimens. Our laboratory uses an Epstein-Barr virus (EBV)-transformed lymphoblastoid cell line (LCL) to measure in vitro radiosensitivity (Figure 2).

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