Hepatic
sinusoidal obstruction syndrome is frequently linked to high-dose
chemotherapy/total-body irradiation in recipients of haematopoietic
stem cell transplantation, long-term use of
azathioprine after
organ transplantation and other chemotherapeutic agents. The incidence of hepatic
sinusoidal obstruction syndrome varies from 0% to 70%, and is decreasing. Disease risk is higher in patients with
malignancies, hepatitis C virus
infection, those who present late, when
norethisterone is used to prevent menstruation, and when broad-spectrum
antibiotics and antifungals are used during and after the conditioning therapy. Hepatic
sinusoidal obstruction syndrome presents with tender
hepatomegaly, hyperbilirubinaemia and
ascites, and diagnosis is mainly clinical (Seattle and Baltimore Criteria). Imaging excludes biliary obstruction and
malignancy, but cannot establish accurate diagnosis. Hepatic
sinusoidal obstruction syndrome may be prevented by avoiding the highest risk regimens, using non-myelo-ablative regimens, and reducing total-body irradiation dose. Treatment is largely symptomatic and supportive, because 70-80% of patients recover spontaneously.
Tissue plasminogen activator plus
heparin improves outcome in <30% of cases.
Defibrotide, a
polydeoxyribonucleotide, is showing encouraging results. Transjugular intrahepatic porto-systemic shunt relieves
ascites, but does not improve outcome.
Liver transplantation may be an option in the absence of
malignancy. Prognosis is variable and depends on disease severity, aetiology and associated conditions. Death is most commonly caused by renal or cardiopulmonary failure.