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Scholarly Research Exchange / 2009 / Article

Case Report | Open Access

Volume 2009 |Article ID 974576 | 4 pages | https://doi.org/10.3814/2009/974576

Hodgkin's Disease with Atypical Clinical Presentation, Associated with Ataxia-Telangiectasia

Received04 Mar 2009
Revised03 May 2009
Accepted12 May 2009
Published02 Jun 2009

Abstract

The authors report a 4-year-old male with Hodgkin's disease with atypical clinical presentation, laboratory data and imaging studies as well as an increased radiosensitivity. These were the first symptoms which raised the suspicion of ataxia-telangiectasia. The lymphoma presented as a solid tumor mass in the right upper posterior mediastinum without peripheral lymphadenopathy. The alpha-fetoprotein was significantly increased and the diagnosis of a germ-cell tumor or neuroblastoma was suspected. After partial resection of the tumor the histology indicated Hodgkin's disease-mixed cellularity. The boy received chemotherapy according to DAL HD-95 protocol without any unusual toxicity. Low-dose radiation therapy of the upper and middle mediastinum was followed by early radiation-induced esophagitis and appearance of a large pericardial effusion one year later. Ataxia and bulbar and skin telangiectasias at the radiation site appeared in the next years. He suffered recurrent pulmonary infections and succumbed to severe pneumonia with respiratory failure and pulmonary hypertension five years after the diagnosis of malignant lymphoma without evidence of recurrence of Hodgkin's disease.

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Copyright © 2009 M. I. Spasova et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


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