Ataxia-Telangiectasia and CD8+T Cells Acute Lymphoid Leukemia in a Brazilian Patient: A Case Report

Abstract

A Brazilian boy with Ataxia-Telangiectasia (AT) diagnosed at 7 years of age, looked for the hospital at the age of 9 complaining of vomiting and disseminated hematomas for 10 days. The initial evaluation revealed only anemia (Hb=8,3 g/dL) and thrombocytopenia (19000 platelets/mm3). The bone marrow aspirate showed 90% of lymphoid blasts, being diagnosed CD8+ T cells acute leukemia and the patient was submitted to chemotherapy. During the treatment, he developed pneumonia, hepatomegaly and acute tumor lysis syndrome, followed by septic shock and acute renal failure. Despite intensive care efforts, he died a month after cancer diagnosis. AT is a neurodegenerative disease, associated to immunodeficiency, high sensitivity to ionizing radiation and predisposition to cancer. This case revealed how aggressive could be a neoplasia in AT patients, in which a careful cancer surveillance is mandatory, with clinical and laboratorial control, avoiding unnecessary radiation exposure.