We report a new case of ataxia-telangiectasia (AT) and acute lymphoblastic leukemia (ALL) and review all 21 known cases of AT and ALL. Leukemia in these patients is associated with a male predominance, age older than 10 years at diagnosis, a white blood cell count higher than 50,000 mm3, and a fatal course. Four patients have been reported who developed T-cell leukemia, 3 null-cell leukemia and 1 B-cell leukemia. The AT-ALL patients appear to be at risk for infections related to their immunodeficient status and ALL chemotherapy. In addition, neurologic deterioration has been noted during the early phase of therapy.
Get full access to this article
View all access options for this article.
References
1.
BiggarW.D., GoodR.A.: Immunodeficiency in ataxia-telangiectasis. In Birth Defects: Original Article Series, Vol. XI. pp. 271–276. Baltimore, Williams and Wilkins, 1975.
2.
HechtF., KolerR.D., RigasD.A., DahukeG.S., CaseM.P., TisdaleV., MillerR.W.: Leukemia and lymphocytes in ataxia-telangiectasia.Lancet, 2: 1193, 1966.
3.
KerseyJ.H., SpectorB.D., GoodR.A.: Primary immunodeficiency diseases and cancer: The immunodeficiency Cancer Registry.Int. f. Cancer, 12: 333–347, 1973.
SpectorB.D., PerryG.S., KerseyJ.H.: Genetically determined immunodeficiency diseases and malignancy: Report from the Immunodeficiency Cancer Registry.Clin. Immunol. and Immunopathol., 11: 12–29, 1978.
6.
SwiftM.: Malignant disease in heterozygous carriers. In Birth Defects: Original Article Series, Vol. XII, pp. 133–144. Alan R. Liss, New York, 1975.
7.
ToledanoS.T., LangeB.J.: Ataxia-telangiectasia and acute lymphoblastic leukemia.Cancer, 45: 1675–1678, 1980.
8.
WeichselbaumR.R., LittleJ.: Familial retinoblastoma and ataxia telangiectasia: Human models for the study of DNA damage and repair.Cancer45: 775–779, 1980.
