Table of Contents
Scholarly Research Exchange
Volume 2009, Article ID 974576, 4 pages
http://dx.doi.org/10.3814/2009/974576
Case Report

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

1Department of Pediatrics, Medical University-Plovdiv, 4000 Plovdiv, Bulgaria
2Department of Oncology and Hematology, Medical University-Plovdiv, 4000 Plovdiv, Bulgaria

Received 4 March 2009; Revised 3 May 2009; Accepted 12 May 2009

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.

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.