“Pre-emptive strike”-the case for early treatment of hepatic sinusoidal obstruction syndrome with defibrotide
Document Type
Article
Publication Date
1-1-2018
Abstract
The initial signs of hepatic sinusoidal obstruction syndrome (HSOS) can be challenging to recognize in children, especially outside the hematopoietic stem cell transplantation setting. To assist clinicians to promptly identify HSOS, the European Society for Blood and Marrow Transplantation has proposed pediatric HSOS diagnostic criteria which emphasize unexplained consumptive and transfusion-refractory thrombocytopenia. To highlight the importance of these “bellwether” early signs of HSOS and the efficacy of pre-emptive treatment with defibrotide, we describe the case of a child with a right 11th rib primitive neuroectodermal tumor who developed HSOS following focal radiotherapy and actinomycin-D treatment.
Keywords
actinomycin-D, defibrotide, hepatic sinusoidal obstruction syndrom, eradiotherapy, thrombocytopenia
Divisions
fac_med
Funders
Cancer Council of Western Australia
Publication Title
Pediatric Blood & Cancer
Volume
65
Issue
7
Publisher
Wiley