T-CELL LEUKEMIA REVEALING ATAXIA-TELANGIE CTASIA

Background. - Variable degrees of T cell deficiency in ataxia-telangie ctasia (AT) progressively worsen with time and death from malignant ly mphoma is a common terminal event. T-cell lymphoma as the first manife station of AT has never been reported Case report. - A 22 month-old gi rl born to consanguineous parents, was treated for a thoracic T-cell l ymphoma and remained in first complete remission, with a follow-up of 4 years, Prior to chemotherapy, cytogenetic studies on blood showed cl onal rearrangements including t(7p;14q), T(2p;7q) and inv (7), while k aryotype showed 6q- and 1p-mitoses on bone marrow blasts. Hypotonia be came evident at 3 years. One year later, the neurological status deter iorated. The patient presented also severe respiratory tract infection s. At that time, immunological investigations showed hypo IgG2, very l ow T4 lymphocytes level, all harbouring the CD45 RO phenotype. Increas e in alpha-foetoprotein level, the ocular movements and the study of D NA synthesis after exposure to grays confirmed the diagnosis of AT. Co nclusion. - In cases of childhood lymphoid neoplasia, AT should be con sidered whenever parental consanguinity, T-cell proliferation and/or u nexpected toxic therapeutic responses are noted.