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Non‐activated protein C rescue treatment in Wilms tumour associated hepatic sinusoidal obstructive syndrome

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.