Skip Navigation LinksHome > May 2014 - Volume 36 - Issue 4 > Liver Failure Due to Hepatic Angiosarcoma in an Adolescent W...
Journal of Pediatric Hematology/Oncology:
doi: 10.1097/MPH.0b013e318286d4d4
Clinical and Laboratory Observations

Liver Failure Due to Hepatic Angiosarcoma in an Adolescent With Dyskeratosis Congenita

Olson, Timothy S. MD, PhD*,†; Chan, Elaine S. MD; Paessler, Michele E. DO; Sullivan, Kathleen E. MD, PhD§; Frantz, Christopher N. MD; Russo, Piere MD; Bessler, Monica MD, PhD*,¶

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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.

Copyright © 2013 by Lippincott Williams & Wilkins

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