Sinusoidal Obstruction Syndrome (Veno-occlusive Disease) Following Hematopoietic Stem Cell Transplant: Insights and Therapeutic Advances

Abstract

Hepatic sinusoidal obstruction syndrome (SOS) is a rare fatal clinical entity seen following hematopoietic stem cell transplant (HSCT). It is more commonly reported to occur following allogeneic HSCT compared to autologous HSCT. Historically, it is known as hepatitis following HSCT. It is thought that endothelial damage to the hepatic venules leading to occlusion of the terminal hepatic venules and hepatic sinusoids is the trigger for the development of SOS. Several risk factors have been associated with this condition. Some of these risk factors are patient related while others are transplant process related. Given the high mortality of this condition, early identification of high-risk patients with severe disease is of utmost importance. The management of SOS varies depending on the severity of the disease. Mild to moderate disease has a good outcome with supportive measures alone, while severe presentation of the disease requires a more aggressive management. Defibrotide is the only Food and Drug Administration-approved therapy and it is reserved for severe cases of SOS. The role of defibrotide as a prophylactic therapy remains under investigation.

Liver transplantation following late‐onset hepatic sinusoidal obstruction syndrome occurred beyond 1‐year postallogeneic hematopoietic stem cell transplantation

“… 9 In the literature, MRI scans of HVOD showed hepatomegaly, ascites, hepatic vein narrowing, gallbladder wall thickening, periportal cuffing, or patchy signal enhancement of the liver. 10 , 11 , 12 The MRI of this patient presented similar imaging findings suggesting the possibility of HVOD. Histological evidence of HVOD remains the gold standard (but not mandatory) for the diagnosis.…”

Development of a Drum Tower Severity Scoring (DTSS) system for pyrrolizidine alkaloid-induced hepatic sinusoidal obstruction syndrome

“…In the West, HSOS is often observed following the high-dose chemotherapy used in hematopoietic stem cell transplantation (HSCT). Toxic metabolites arising from chemotherapy used in HSCT can lead to the injury of sinusoidal endothelial cells and hepatocytes, and the obstruction of the hepatic sinusoids and terminal hepatic veins, thus promoting the development of hepatic dysfunction and acute portal hypertension [ 16 , 17 ]. Previous studies have shown that a history of prior liver disease and hematologic disorders are high risk factors for HSOS progression [ 8 ].…”

Anticoagulant therapy likely increases risk of bleeding in Gynura segetum-induced hepatic sinus obstruction syndrome

“…Some researchers have reported the use of unfractionated or low-molecular-weight heparin for the treatment of Tusanqi-induced HSOS with success rates of 70% to 88.9%. [4][5][6][7] Anticoagulant therapy is associated with serious complications such as bleeding, which renders its use controversial. Therefore, here we retrospectively analyzed the clinical characteristics of 49 patients with Tusanqi-induced HSOS as well as the benefits and risks associated with anticoagulant therapy.…”