Garita MK, Rodríguez RC

Language: Spanish
References: 14
Page: 623-628
PDF size: 455.45 Kb.

ABSTRACT

Ataxia telangiectasia is a primary immunodeficiency and, at the same time, one of the cancer predisposition syndromes. The incidence of cancer in patients with ataxia telangiectasia is approximately 100-fold greater than expected for an age-matched population. Ataxia telangiectasia patients are unusually sensitive to ionizing radiation and some radiomimetic chemotherapy agents. For them, treatment of cancer with conventional doses of radiation can be fatal. We present the case of an eleven years old male patient with ataxia telangiectasia and with diagnosis of Hodgkin’s Lymphoma when he was four years old. The first chemotherapeutic protocol chosed for this boy was interrupted many times because of recurrent respiratory tract infections. This probably was associated, in a good part, with the persistence of his disease. The second chemotherapeutic protocol was better tolerated, the compliance of the treatment was higher and the child achieved complete remission; he was free of disease for a period of six years. Nevertheless, this last protocol establishes that it is necessary to complete management with radiotherapy, but it wasn’t possible in our patient because of the presence of ataxia telangiectasia. Finally, the child died. Literature describes a very poor prognosis in patients with ataxia telangiectasia and Hodgkin’s lymphoma. This has been attributed to advanced state of the disease, difficulties to recognize the presence of concomitant chronic lung disease and the lack of standarization in the regimens of treatment used.

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