Renal Lymphoma in Ataxia-Telangiectasia

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Abstract
Ataxia-telangiectasia (AT) or the Louis-Bar syndrome is a familial, multisystem disorder. Patients with the disease are at high risk for malignancy, in particular lymphoreticular neoplasms, and immunodeficiency, rendering them susceptible to infection. We present a 17-year-old girl with AT and a history of lymphoma who developed a persistent fever. An isolated renal mass was discovered by abdominal ultrasound and CT. Although the diagnosis of renal lymphoma is strongly suggested by these modalities, other types of renal mass lesions, including renal infection, may occur in patients with AT and ultimately a definitive diagnosis may only be established by biopsy.

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