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Hepatic sinusoidal obstruction syndrome: 5 case reports A retrospective study of 82 children with various cancer, who underwent autologous stem cell transplant (ASCT) at Royal Marsden Hospital, United Kingdom, between March 2010 and February 2017, described five children [sexes and exact ages not stated], who developed hepatic sinusoidal obstruction syndrome (SOS) following conditioning treatment with busulfan or melphalan [not all routes and dosages stated; durations of treatments to reaction onsets not stated]. Five children with unspecified malignancy (4 patients) or relapsed Wilms tumour (1 patient) underwent ASCT at a hospital in United Kingdom. Four of these children received conditioning regimen comprising IV busulfan divided in 16 doses followed by (after 24 hours) IV melphalan 140 mg/m2, while the remaining child received conditioning regimen with high dose melphalan. Two of the children form busulfan plus melphalan regimen group received defibrotide prophylaxis in divided doses daily from the start of conditioning regimen. Additionally, all children received prophylactic ursodeoxycholic acid. 16–20 days after ASCT, the children were diagnosed with moderate–very severe hepatic SOS (n=5). Multiple organ dysfunction including renal and pulmonary involvement developed in the child with very severe SOS. Doppler studies showed reversal of venous flow in only one child. Peak bilirubin levels were 35–102 µmol/L. Two children required admission to paediatric ICU. All children received treatment with defibrotide and ursodeoxycholic acid. Additionally, the children received fluid restriction and unspecified diuretics were given to control fluid overload/positive fluid balance and weight gain. All children required transfusion support. The child with very severe SOS required transfusion of albumin and plasma/cryoprecipitate along with renal replacement therapy and ventilation. The SOS resolved after 13–28 days from onset. The total duration of hospitalisation was 40–54 days. All children survived. Roy Moulik N, et al. Defibrotide treatment but not prophylaxis is useful in hepatic sinusoidal obstruction syndrome in children undergoing autologous stem cell transplant following high-dose chemotherapy: A single-center experience from the Royal Marsden Hospital, UK. Pediatric Blood and Cancer 67: No. 11, Nov 2020. Available from: 803519086 URL: http://doi.org/10.1002/pbc.28677

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Reactions 5 Dec 2020 No. 1833