Abstract
Hepatic veno-occlusive disease (VOD) or sinusoidal obstruction syndrome (SOS) is a rare complication characterized by hepatomegaly, right-upper quadrant pain, jaundice, and ascites, occurring after high-dose chemotherapy, hematopoietic stem cell transplantation (HSCT) and, less commonly, other conditions. We review pathogenesis, clinical appearance and diagnostic criteria, risk factors, prophylaxis, and treatment of the VOD occurring post-HSCT. The injury of the sinusoidal endothelial cells with loss of wall integrity and sinusoidal obstruction is the basis of development of postsinusoidal portal hypertension responsible for clinical syndrome. Risk factors associated with the onset of VOD and diagnostic tools have been recently updated both in the pediatric and adult settings and here are reported. Treatment includes supportive care, intensive management, and specific drug therapy with defibrotide. Because of its severity, particularly in VOD with associated multiorgan disease, prophylaxis approaches are under investigation. During the last years, decreased mortality associated to VOD/SOS has been reported being it attributable to a better intensive and multidisciplinary approach.
Highlights
Hepatic veno-occlusive disease (VOD), known as sinusoidal obstruction syndrome (SOS), is a clinical syndrome occurring after high-dose chemotherapy, hematopoietic stem cell transplantation (HSCT) [1, 2], and, less commonly, after ingestion of toxic alkaloids [3], after high doses of radiotherapy [4] or liver transplantation [5]
Clinical appearance and diagnostic criteria, risk factors, prophylaxis, and treatment of the VOD occurring post-HSCT
The initial step of VOD/SOS pathogenesis is the injury of sinusoidal endothelium of the liver (Figure 1) leading to loss of endothelial cell cohesions: gaps appear in the endothelial barrier, and red blood cells pass through these gaps and accumulates in the Disse space, causing the detachment of the endothelial cells with downstream embolization of the centrilobular vein and subsequent postsinusoidal obstruction [18]
Summary
Hepatic veno-occlusive disease (VOD), known as sinusoidal obstruction syndrome (SOS), is a clinical syndrome occurring after high-dose chemotherapy, hematopoietic stem cell transplantation (HSCT) [1, 2], and, less commonly, after ingestion of toxic alkaloids (toxic injury) [3], after high doses of radiotherapy [4] or liver transplantation [5]. The initial step of VOD/SOS pathogenesis is the injury of sinusoidal endothelium of the liver (Figure 1) leading to loss of endothelial cell cohesions: gaps appear in the endothelial barrier, and red blood cells pass through these gaps and accumulates in the Disse space, causing the detachment of the endothelial cells with downstream embolization of the centrilobular vein and subsequent postsinusoidal obstruction [18]. Incidence of VOD/SOS is higher after allogenic compared to autologous HSCT and is higher in patients receiving a transplant from a mismatched unrelated donor [31]. These observations are supported by findings in experimental models where endothelial cells are targets of alloreactive T cells [32]. Endothelial cell detachment seems to be correlated with nitric oxide deficiency caused by postconditioning toxicity [36]
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