Introduction: Veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is a potentially life-threatening condition characterised by obstruction of the small veins of the liver. Although typically associated with haematopoietic stem cell transplantation, VOD/SOS may also occur following intensive multimodal chemotherapy regimens. In children, symptoms of VOD/SOS are refractory thrombocytopaenia, weight gain, hepatomegaly, ascites and fluid retention, hyperbilirubinaemia and sometimes right upper quadrant pain.

Methods: Here, we present a case series of six paediatric patients with acute lymphoblastic leukaemia who developed severe VOD/SOS while receiving standard AIEOP-BFM ALL protocol treatment.

Results/conclusions: All patients responded promptly to defibrotide treatment and exhibited favourable clinical outcomes.

Trial Registration: The authors have confirmed clinical trial registration is not needed for this submission.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11756966PMC
http://dx.doi.org/10.1002/jha2.1094DOI Listing

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Introduction: Veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is a potentially life-threatening condition characterised by obstruction of the small veins of the liver. Although typically associated with haematopoietic stem cell transplantation, VOD/SOS may also occur following intensive multimodal chemotherapy regimens. In children, symptoms of VOD/SOS are refractory thrombocytopaenia, weight gain, hepatomegaly, ascites and fluid retention, hyperbilirubinaemia and sometimes right upper quadrant pain.

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Sinusoidal obstruction syndrome (SOS), also known as hepatic veno-occlusive disease (VOD), is a life-threatening complication of hematopoietic stem cell transplantation. In severe cases, SOS/VOD progresses to multiple organ failure with a mortality rate higher than 80%. Early diagnosis and treatment based on severity assessment improve the prognosis of severe SOS/VOD, but conventional diagnostic criteria may be insufficient for an early diagnosis.

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