Catheter-directed thrombolysis and thrombectomy for the Budd-Chiari syndrome in paroxysmal nocturnal hemoglobinuria in three patients

J Vasc Interv Radiol. 2006 Feb;17(2 Pt 1):383-7. doi: 10.1097/01.RVI.0000196338.87954.CE.

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.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adult
  • Budd-Chiari Syndrome / drug therapy*
  • Budd-Chiari Syndrome / etiology
  • Budd-Chiari Syndrome / surgery*
  • Catheterization
  • Combined Modality Therapy
  • Female
  • Hemoglobinuria, Paroxysmal / complications
  • Hemoglobinuria, Paroxysmal / drug therapy*
  • Hemoglobinuria, Paroxysmal / surgery*
  • Humans
  • Male
  • Phlebography
  • Thrombectomy
  • Thrombolytic Therapy