Prophylactic, preemptive, and curative treatment for sinusoidal obstruction syndrome/veno-occlusive disease in adult patients: a position statement from an international expert group

著者: Mohamad Mohty, Florent Malard, Manuel Abecasis, Erik Aerts, Ahmed Alaskar, Mahmoud Aljurf, Mutlu Arat, Peter Bader, Frédéric Baron, Grzegorz Basak, Ali Bazarbachi, Didier Blaise, Fabio Ciceri, Selim Corbacioglu, Jean‐Hugues Dalle, Fiona L. Dignan, Takahiro Fukuda, Anne Huynh, Jürgen Kuball, Silvy Lachance, Hillard M. Lazarus, Tamás Masszi, Mauricette Michallet, Arnon Nagler, Mairéad NíChonghaile, Shinichiro Okamoto, Antonio Pagliuca, Christina Peters, Finn Bo Petersen, Paul G. Richardson, Tapani Ruutu, Wael Saber, Bipin N. Savani, Robert J. Soiffer, Jan Styczyński, Elisabeth Wallhult, Ibrahim Yakoub‐Agha, Rafael F. Duarte, Enric Carreras - Bone Marrow Transplantation 2019 被引用: 112

Sinusoidal obstruction syndrome (SOS), formerly called veno-occlusive disease (VOD; referred to as SOS/VOD hereafter), is a life-threatening complication that can occur after hematopoietic stem cell transplantation (HCT) [ 1 ]. The conditioning regimen and immune-mediated injury following allogeneic HCT (allo-HCT) generate toxic metabolites that damage sinusoidal endothelial cells. The expression of tissue and von Willebrand factors contribute to the clothing cascade activation, perpetuate the endothelial cell injury leading to the formation of gaps in the hepatic sinusoidal endothelium [ 2 ]. Red blood cells penetrate through those gaps in the perisinusoidal space, beneath the endothelial cells, and subsequently dissect off the endothelial lining, all of which embolize as part of the sinusoid flow and in turn obstruct the sinusoid [ 1 ]. This process reduce hepatic outflow, produces postsinusoidal hypertension with tissue ischemia in zone 3 of the acinus, and concomitant hepatocellular damage; all of which results in the clinical symptoms of SOS/VOD and an associated hepato-renal syndrome, namely fluid retention, ascites, weight gain, painful hepatomegaly, and jaundice [ 3 , 4 , 5 ]. In the most severe cases, patients may develop multiorgan dysfunction (MOD) with pulmonary and renal involvement, encephalopathy and, ultimately, death. Despite the incidence of SOS/VOD being limited, around 10–15% after myeloablative allo-HCT and up to 5% after reduced-intensity conditioning (RIC) allo-HCT, particular attention must be paid to permit its early detection and treatment and to prevent the development of the most severe forms, which are in turn associated with a very high mortality rate (>80%) [ 1 , 5 ]. In an effort to improve early diagnosis, the European Group for Blood and Marrow Transplantation (EBMT) revise the modified Seattle [ 6 ] and Baltimore [ 4 ] criteria, and recently published revised diagnosis and severity criteria for adults [ 7 ].