title: Catheter-directed thrombolysis and thrombectomy for the Budd-Chiari syndrome in paroxysmal nocturnal hemoglobinuria in three patients.
abstract: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematopoietic stem cell disorder characterized by hemolytic anemia, hemoglobinuria, bone marrow failure, and hypercoagulability. Thrombosis is the leading cause of mortality and occurs in one-half of PNH patients, with the hepatic veins being the most common site. Patients with hepatic vein thrombosis (Budd-Chiari syndrome) can present with abdominal pain, hepatomegaly, jaundice, and ascites. Prognosis is poor for these patients; death may occur from liver failure, vessel rupture, intestinal ischemia, infarction, necrosis, or sepsis. The authors report three consecutive cases of successful treatment with catheter-directed thrombolysis and thrombectomy directly in the hepatic veins in patients with PNH who developed acute hepatic vein thrombosis. This treatment represents a potential bridge toward more curative therapies such as allogeneic bone marrow transplant.
authors.affiliation: Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University School of Medicine, Baltimore, MD 21287-4010, USA.
authors.forename: George P
authors.initials: GP
authors.lastname: Kuo
authors.forename: Robert A
authors.initials: RA
authors.lastname: Brodsky
authors.forename: Hyun S
authors.initials: HS
authors.lastname: Kim
mesh_terms: D000293:Adolescent; D000328:Adult; D006502:Budd-Chiari Syndrome / Q000188:drug therapy* / Q000209:etiology / Q000601:surgery*; D002404:Catheterization; D003131:Combined Modality Therapy; D005260:Female; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications / Q000188:drug therapy* / Q000601:surgery*; D006801:Humans; D008297:Male; D010690:Phlebography; D017131:Thrombectomy; D015912:Thrombolytic Therapy