Hepatic Sinusoidal Obstruction Syndrome in the Era of Defibrotide
Makaleler > Tam Makale · 2017
Yazarlar: Güray SAYDAM
Annals of Hematology & Oncology, Uluslararası, 2017, cilt 4
DOI: 10.26420/annhematoloncol.2017.1131
Özet
Background: Hepatic sinusoidal obstruction syndrome (SOS) termed as hepatic veno-oclusive disease previously (VOD), is a toxic injury of liver sinusoids resulting obliterative venulitis of the terminal hepatic venules.In hematopoietic stem cell transplantation (HSCT) setting, SOS appears at a rate of 10 to 60% and severe SOS with hepatic failure causes considerable mortality.Case Report: A 40-year-old male with myelodysplastic syndrome was underwent HSCT from matched unreleated donor in active disease status.Hepatic SOS was diagnosed on 21th day of HSCT with epigastric pain and abdominal distension.The patient was successfully treated with defibrotide and steroids.Conclusion: Defibrotide, a novel agent in SOS, has been shown to improve the clinical outcome and survival in patients with severe VOD.Avoiding from major risk factors is primary method of preventing from SOS. Patients identified as being at higher risk for SOS and the risk could not be decreased, should be considered for DF prophylaxis.