Ataxia-telangiectasia and acute lymphoblastic leukemia

Abstract

Lymphoreticular malignancies occur frequently in association with ataxia-telangiectasia (A-T). This paper presents one new case and reviews all 19 known cases of A-T and acute lymphoblastic leukemia (ALL). The leukemia is associated with poor prognostic factors: male sex, older age, high white blood cell count, and T or B-cells. There have been no long-term survivors; recently, prolonged remissions have been attained. These patients appear to be at special risk for infections secondary to the immunodeficient state associated with A-T and ALL therapy. A heightened sensitivity to the neurotoxic effects of vinca alkaloids and central nervous system irradiation seems to be present.

Hodgkin lymphoma in a young child contributing to a diagnosis of ataxia telangiectasia: review of the literature

“…Conversely, most cases of acute lymphoblastic leukemia in A-T present at an older age (average 9 years) [18]. Only one case of acute myeloid leukemia in an A-T patient has been reported in the [19].…”

The teenage coeliac: follow up study of 102 patients.

“…Such interpolated data were transformed into standard deviation scores (SDS) according to Tanner's formula SDS=(xe-Xm)/SDm where xe is the measurement at the relevant age and Xm and SDm are normal mean and normal standard deviation at the age in question, respectively (see table). Means 4 It would be interesting, in the Newcastle case, to know the temporal distribution of the reported 'gaps and reductions' in methotrexate/6-mercaptopurine treatment. If most occurred during the first year of treatment, rather than the second, it may be that lingering radiation treatment induced DNA damage to bone marrow in the craniocervical area, rather than chemotherapy, was blameworthy.…”

Leukoencephalopathy after prophylactic radiation for leukaemia in ataxia telangiectasia.

“…A RADZIKOWSKI, T 4 It would be interesting, in the Newcastle case, to know the temporal distribution of the reported 'gaps and reductions' in methotrexate/6-mercaptopurine treatment. If most occurred during the first year of treatment, rather than the second, it may be that lingering radiation treatment induced DNA damage to bone marrow in the craniocervical area, rather than chemotherapy, was blameworthy.…”