Sinusoidal Obstruction Syndrome/Veno-occlusive disease (SOS/VOD) in Japan

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  • 本邦における肝類洞閉塞症候群/肝中心静脈閉塞症

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<p> Sinusoidal obstruction syndrome/veno-occlusive disease (SOS/VOD) is known to be one of lethal complications after hematopoietic stem cell transplantation (HSCT). In Japan, 462 of 4,290 patients who underwent allogeneic HSCT between 1999 and 2010 were diagnosed with SOS/VOD according to the Seattle criteria (cumulative incidence 10.8%). The 100 day overall survival rate was 32%. Multivariate analyses showed that the statistically significant independent risk factors for SOS/VOD were the number of HSCTs, age, performance status, hepatitis C virus-seropositivity, advanced disease status, and myeloablative regimen. SOS/VOD was highly associated with overall mortality. Defibrotide (DF) has been approved and become available in Japan. Recombinant human soluble thrombomodulin (rhTM) is approved for the treatment of disseminated intravascular coagulation, which pathologically resembles SOS/VOD in the etiology associated with endothelial damage. Data of 65 patients who underwent allogeneic HSCT and received DF (n=24) or rhTM (n=41) for SOS/VOD treatment were collected. Complete response rates for SOS/VOD on day 100 were 50% and 54% in the DF and rhTM groups, respectively. The 100 day overall survival rates were 50% in the DF group and 48% in the rhTM group, respectively. Although we can use defibrotide for SOS/VOD treatment globally, SOS/VOD remains one of the critical problems.</p>

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