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Non‐activated protein C rescue treatment in Wilms tumour associated hepatic sinusoidal obstructive syndrome
Author(s) -
De Leonardis Francesco,
Koronica Roberta,
Bruno Stefania D.,
Santoro Nicola
Publication year - 2014
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.24859
Subject(s) - medicine , blood cancer , wilms' tumor , chemotherapy , complication , supportive psychotherapy , obstructive jaundice , haematopoiesis , cancer , stem cell , gastroenterology , oncology , biology , genetics
Hepatic sinusoidal obstructive syndrome (HSOS) is a frequent complication in patients undergoing haematopoietic stem cell transplant (HSCT), and more rarely, in paediatric patients receiving conventional chemotherapy for solid tumours. Its diagnosis relies on a combination of clinical signs and symptoms such as hepatomegaly, jaundice, weight gain and fluid retention. HSOS treatment is primarily based on supportive care and anti‐fibrinolytic agents. Here we report two patients affected by Wilms tumour who developed life‐threatening HSOS that failed to respond to conventional treatment. Both patients recovered after receiving aggressive supportive treatment that included administration of non‐activated protein C (Ceprotin®—Baxter). Pediatr Blood Cancer 2014;61:940–941. © 2013 Wiley Periodicals, Inc.