Hepatic veno-occlusive disease (VOD), also known as sinusoidal obstruction syndrome, is a major complication of hematopoietic stem cell transplantation and it carries a high mortality. Prophylaxis for hepatic VOD is commonly given to transplant recipients from the start of conditioning through the early weeks of transplant. However, high quality evidence from randomized controlled trials is scarce with small sample sizes and the trials yielded conflicting results. Although various treatment options for hepatic VOD are available, most have not undergone stringent evaluation with randomized controlled trial and therefore it remains uncertain which treatment offers real benefit. It remains controversial whether VOD prophylaxis should be given, which prophylactic therapy should be given, who should receive prophylaxis, and what treatment should be offered once VOD is established.

• Hepatic veno-occlusive disease
• Hematopoietic stem cell transplantation
• Prophylaxis
• Treatment
• Randomized controlled trial

Our goal is to provide a detailed review of veno-occlusive disease (VOD), Budd-Chiari syndrome (BCS), and congestive hepatopathy (CH), all of which results in hepatic venous outflow obstruction. This is the first article in which all three syndromes have been reviewed, enabling the reader to compare the characteristics of these disorders. The histological findings in VOD, BCS, and CH are almost identical: sinusoidal congestion and cell necrosis mostly in perivenular areas of hepatic acini which eventually leads to bridging fibrosis between adjacent central veins. Tender hepatomegaly with jaundice and ascites is common to all three conditions. However, the clinical presentation depends mostly on the extent and rapidity of the outflow obstruction. Although the etiology and treatment are completely different in VOD, BCS, and CH; the similarities in clinical manifestations and liver histology may suggest a common mechanism of hepatic injury and adaptation in response to increased sinusoidal pressure.

• Veno-occlusive disease
• Sinusoidal obstru-ction syndrome
• Budd-Chiari syndrome
• Congestive hepatopathy

• Budd-Chiari Syndrome/etiology
• Budd-Chiari Syndrome/pathology
• Budd-Chiari Syndrome/therapy
• Hepatic Veno-Occlusive Disease/etiology
• Hepatic Veno-Occlusive Disease/pathology
• Hepatic Veno-Occlusive Disease/therapy
• Humans
• Liver Diseases/etiology
• Liver Diseases/pathology
• Liver Diseases/therapy
• Necrosis/pathology
• Prognosis
• Syndrome

• Diagnosis, Differential
• Hematopoietic Stem Cell Transplantation/adverse effects
• Hepatic Veno-Occlusive Disease/classification
• Hepatic Veno-Occlusive Disease/diagnosis
• Hepatic Veno-Occlusive Disease/etiology
• Hepatic Veno-Occlusive Disease/pathology
• Hepatic Veno-Occlusive Disease/therapy
• Humans
• Prognosis
• Risk Factors
• Severity of Illness Index

• Antineoplastic Combined Chemotherapy Protocols/adverse effects
• Hematopoietic Stem Cell Transplantation/adverse effects
• Hepatic Veno-Occlusive Disease/drug therapy
• Hepatic Veno-Occlusive Disease/etiology
• Hepatic Veno-Occlusive Disease/prevention & control
• Humans