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Liver failure due to hepatic angiosarcoma in an adolescent with dyskeratosis congenita.

Abstract
Dyskeratosis congenita (DC) is a multisystem disease caused by genetic mutations that result in defective telomere maintenance. Herein, we describe a 17-year-old patient with severe DC, manifested by bone marrow failure, severe immunodeficiency, and enterocolitis requiring prolonged infliximab therapy, who developed fatal hepatic failure caused by an aggressive, infiltrating hepatic angiosarcoma. Although DC patients have known increased risk of developing liver failure and multiple types of malignancy, this report is the first to describe angiosarcoma in a DC patient. Malignancy should thus be considered in the differential diagnosis of progressive liver dysfunction in DC patients.
AuthorsTimothy S Olson, Elaine S Chan, Michele E Paessler, Kathleen E Sullivan, Christopher N Frantz, Piere Russo, Monica Bessler
JournalJournal of pediatric hematology/oncology (J Pediatr Hematol Oncol) Vol. 36 Issue 4 Pg. 312-5 (May 2014) ISSN: 1536-3678 [Electronic] United States
PMID23588325 (Publication Type: Case Reports, Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)
Topics
  • Adolescent
  • Dyskeratosis Congenita (complications, pathology)
  • Hemangiosarcoma (etiology, pathology)
  • Humans
  • Liver Failure (etiology, pathology)
  • Liver Neoplasms (etiology, pathology)
  • Male

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