Clinical characteristics and treatment of pyrrolizidine alkaloid-related hepatic vein occlusive disease.

Abstract

Hematopoietic stem cell transplantation related hepatic vein occlusive disease (HSCT-HVOD) has been researched extensively; however, little is known about the clinical features and treatment of pyrrolizidine alkaloid-induced HVOD (PA-HVOD). This retrospective single centre study examined the clinical and laboratory characteristics of 108 patients with acute PA-HVOD and explored the efficacy of anticoagulation and TIPS therapy. The study included 108 consecutive patients with PA-HVOD between July 2008 and June 2016. The clinical manifestations and the results of laboratory and imaging tests were evaluated. The survival rates of patients treated with different approaches were recorded. Serum total bilirubin was <34.2μmol/L (2mg/dL) in approximately 40% of patients. More than 90% of patients were presented with hepatomegaly, uneven liver perfusion in the balance phase, compressive stenosis of the hepatic segmental inferior vena cava and decreased peak velocity of portal vein blood flow. Severe portal hypertension was observed in all patients undergoing HVPG examination or TIPS operation. Anticoagulation therapy with low molecular weight heparin combined with warfarin was significantly more effective than liver protection and supportive therapy, and TIPS further improved the prognosis of patients who did not respond to anticoagulation therapy. The total effective rate of the anticoagulation-TIPS ladder therapeutic strategy was 91%. Patients with PA-HVOD had different characteristics than those with HSCT-HVOD. Anticoagulation and TIPS treatment may be effective for patients with PA-HVOD.