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KMID : 0387820160230020184
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Clinical Pediatric Hematology-Oncology
2016 Volume.23 No. 2 p.184 ~ p.187
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A Case of Hodgkin Lymphoma Developed in an Ataxia-telangiectasia Patient
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Lim Ju-Yeon
Jeon Mi-Na
Kim Hyo-Sun
Han Jung-Woo
Lyu Chuhl-Joo
Hahn Seung-Min
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Abstract
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Ataxia-telangiectasia (AT) is characterized by cerebellar ataxia, progressive immunodeficiency, radiation sensitivity, telangiectasia, and predisposition to malignancy. AT patients have a 100-fold increased risk for the development of lymphoid malignancies. It is important to consider AT in a child with pre-existing ataxia, or lymphoid malignancy that was diagnosed at a younger age than expected. This consideration avoids the confusion between ataxia development and toxicity from chemotherapy. Hodgkin’s lymphoma (HL) is usually treated with chemotherapy and/or radiotherapy. Unfortunately, when treated with conventional doses of radiotherapy, AT patients invariably experience devastating necrosis of their normal tissues. Therefore, a new treatment protocol for patients with HL in AT must be established. In this paper, we report the case of an 8-year-old female patient with HL in AT who was treated with chemotherapy. This patient was also treated with brentuximab (which targets CD30) for salvage therapy after the disease progressed.
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KEYWORD
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Ataxia-telangiectasia, Hodgkin lymphoma, Chemotherapy, Brentuximab
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