Hepatic Veno-occlusive Disease (Sinusoidal Obstruction Syndrome) After Hematopoietic Stem Cell Transplantation

Abstract

Hepatic veno-occlusive disease (VOD), increasingly referred to as sinusoidal obstruction syndrome, is a well-recognized complication of hematopoietic stem cell transplantation and contributes to considerable morbidity and mortality. In the Western Hemisphere, VOD, classified as a conditioning-related toxicity, is most commonly caused by stem cell transplantation. VOD has been described after all types of stem cell transplantation, irrespective of the stem cell source, type of conditioning therapy, or underlying disease. Recognition of this disease in the posttransplantation setting remains a challenge in the absence of specific diagnostic features because many other more common conditions can mimic it. Limited therapeutic or preventive strategies are currently available for the management of VOD. In this review, we provide a comprehensive account of the pathophysiology of this disease as we understand it today, risk factors for its development, and the current state of knowledge regarding preventive and therapeutic options.

The role of the endothelium in the short-term complications of hematopoietic SCT

“…Thus, despite the possibility that other factors can contribute to VOD development, endothelial injury seems to be the initiating event in the cascade of events leading to clinical manifestation of this complication. [5][6][7] These observations of VOD, the fact that the pathogenesis of the remaining early complication seems to have a close relationship with the microvascular tree (Table 1), and their overlapping clinical manifestations (Table 2), are the main reasons these syndromes have been grouped under the denomination of vascular syndromes or vascular endothelial syndromes after HSCT.…”

Successful treatment of sinusoidal obstructive syndrome after hematopoietic stem cell transplantation with recombinant human soluble thrombomodulin

“…1 SOS usually occurs within the first 3 weeks after high-dose chemotherapy followed by hematopoietic stem cell transplantation (HSCT). 1,2 SOS develops in 5-60% of patients after HSCT and is recognized as one of the most common and important regimen-related toxicities, given the high mortality rate.…”

Clinical significance of hemophagocytosis in BM clot sections during the peri-engraftment period following allogeneic hematopoietic SCT

“…ES, sinusoidal obstruction syndrome, intestinal transplant-associated microangiopathy (intestinal TAM), 21,22 and posterior reversible encephalopathy syndrome were categorized as endothelial complications, as they are caused by vascular endothelial damage. 4,5,[21][22][23][24][25] P-values of less than 0.05 were considered statistically significant.…”

“…3 In addition to aGVHD, pro-inflammatory cytokine release is implicated in the pathogenesis of various early complications after allo-HSCT, such as sinusoidal obstruction syndrome, engraftment syndrome (ES) and capillary leakage syndrome. [4][5][6][7] Although the role of macrophages in these complications is undetermined, macrophages have an ability to secrete significant amounts of pro-inflammatory cytokines. 2 Furthermore, fatal outcomes of hemophagocytic syndrome after allo-HSCT have been described in case reports.…”