RT Journal
A1 Haerer AF, Jackson JF, Evers CG
T1 ATaxia-telangiectasia with gastric adenocarcinoma
JF JAMA
JO JAMA
YR 1969
FD December 8
VO 210
IS 10
SP 1884
OP 1887
DO 10.1001/jama.1969.03160360030006
UL http://dx.doi.org/10.1001/jama.1969.03160360030006
AB This communication concerns a large Negro sibship of which five of 12 members had ataxia-telangiectasia. Two affected sisters also had mucinous adenocarcinoma of the stomach in the second decade of life. More detailed studies in one member revealed decreased IgA levels, decreased lymphoblastic transformation index, chromosomal abnormalities, and abnormal thymic and lymphoid tissues, in addition to the typical clinical features of this syndrome. The accepted predisposition to development of lymphoid neoplasms in ataxia-telangiectasia must be extended to include other malignant neoplasms, in view of the occurrence of histologically similar gastric adenocarcinomas in two affected siblings.