ATAXIA-TELANGIECTASIA ASSOCIATED WITH B-CELL LYMPHOMA - THE EFFECT OFA HALF-DOSE OF THE DRUGS ADMINISTERED ACCORDING TO THE ACUTE LYMPHOBLASTIC-LEUKEMIA STANDARD RISK PROTOCOL

Ataxia telangiectasia (A-T) is a rare autosomal recessive disorder cha racterized by cerebellar ataxia, oculocutaneous telangiectasia, and va riable degrees of humoral and cellular immunodeficiency. Affected indi viduals are known to exhibit a high incidence of lymphoma and leukemia . Because of increased chemosensitivity, the treatment of A-T patients with malignancies requires extremely careful planning and caution wit h respect to the we of chemotherapy. The authors rt;bort on a 12-year- old boy with A-T who developed B-cell lymphoma. He received a half-dos e of the drugs administered according to the acute lymphoblastic leuke mia (ALL) protocol issued by our children's cancer study group (9104 S tandard Risk Protocol, Tokai Pediatric Oncology Study Group). As a res ult, he continues to be in complete remission and free of treatment co mplications 32 months after the diagnosis of B-cell lymphoma.