Liver Failure Due to Hepatic Angiosarcoma in an Adolescent with Dyskeratosis Congenita
| Autor: | Elaine S. Chan, Piere Russo, Michele Paessler, Monica Bessler, Kathleen E. Sullivan, Timothy S. Olson, Christopher N. Frantz |
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| Jazyk: | angličtina |
| Rok vydání: | 2014 |
| Předmět: |
Male
Pathology medicine.medical_specialty Adolescent Hemangiosarcoma Malignancy Article Dyskeratosis Congenita medicine Humans Angiosarcoma Immunodeficiency Enterocolitis business.industry Liver Neoplasms Bone marrow failure Hematology medicine.disease Oncology Pediatrics Perinatology and Child Health Differential diagnosis medicine.symptom business Dyskeratosis congenita Liver Failure |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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