Endemic areas have been identified that associate the disease with the consumption of certain teas from plants with pyrrolizidine alkaloids. In industrialized countries, venous occlusive liver disease is often associated with the transplantation of hematopoietic stem cells.
The disease can also occur after chemotherapy or radiotherapy. Both children and adults can be affected. As a result of the occlusion, a painful hepatomegaly, jaundice, and fluid retention, evident in weight gain, dementia, and ascites occur. In the further course, coagulation disorders and hepatic encephalopathy as signs of liver failure as well as impairment of renal function may occur. In terms of differential diagnosis, hepatitis or a graft-versus-host reaction after transplantation may be considered.
As a primary cause, a lesion of the endothelial cells in the liver sinusoids is assumed. This results in non-thrombotic occlusions of the hepatic veins with concentric subendothelial wall thickening. The diagnosis is based on the recognition of the clinical symptoms and their association with a possible cause and the simultaneous exclusion of other causes of liver disease. A liver biopsy may be indicated. Specific therapy is unknown. Anticoagulant drugs are used on a regular basis.
The course of the disease is variable depending on the cause. If, as a result, multiple organ failure or severe bacterial infections occur, mortality is up to 90%.